Apolipoprotein C1 and apoprotein E as potential therapeutic and prognostic targets for adrenocortical carcinoma

Cancer Biomarkers Vol. 42(1): 1-14 @ The Author(s) 2025 Article reuse guidelines: sagepub.com/journals-permissions DOI: 10.1177/18758592241308440 journals.sagepub.com/home/cbm

Sage

IOS Press

Shaojin Li’, Shuixiu Xiao2 and Yongli Situ3 (D

Abstract

Background: Apolipoprotein C1 (APOC1) and Apoprotein E (APOE) play important roles in lipid transport and metab- olism. In recent years, APOC1 and APOE have been shown to play key roles in the occurrence and development of various cancers. However, the expression levels, gene regulatory networks, prognostic values, and target predictions of APOC1 and APOE in adrenocortical carcinoma (ACC) remain unclear.

Methods: Various bioinformatics analysis methods were used, including gene expression profiling interactive analysis, the University of Alabama at Birmingham cancer data analysis portal, biomarker exploration of solid tumors software, the BioPortal for Cancer Genomics, search tool for the retrieval of interacting genes/proteins, gene multiple association net- work integration algorithm, Metascape, transcriptional regulatory relationships unraveled by sentence-based text-mining, LinkedOmics, and genomics of drug sensitivity in cancer analysis.

Results: APOC1 and APOE expression were strongly downregulated in patients with ACC. APOC1 and APOE expression levels were lower in male patients with ACC than those in female patients. Furthermore, APOC1 and APOE expression levels affected the prognosis of patients with ACC. The main functions of APOC1 and its altered neighboring genes (ANG) were organophosphate ester transport, rRNA processing, and positive regulation of cytokine production. Cytolysis, pro- tein ubiquitination, and histone modification were the main functions of APOE and its ANGs. The transcription factor E2F1, tumor protein p53, miR-182, miR-493, Erb-B2 receptor tyrosine kinase 2, and cyclin dependent kinase 1 were key regulatory targets of APOC1, APOE, and the ANGs. APOC1 and APOE expression in patients with ACC were positively associated with immune cell infiltration. Furthermore, anti-programmed cell death protein 1 immunotherapy strongly downregulated the expression of APOC1 in patients with ACC. Both pilaralisib and elesclomol strongly inhibited SW13 cell growth.

Conclusions: This study preliminarily clarified that APOC1 and APOE might be potential therapeutic and prognostic targets for ACC, and identified new targets and treatment strategies for ACC.

Keywords

APOC1, APOE, adrenocortical carcinoma, target prediction, gene regulation network

Received: 25 July 2023; accepted: 5 September 2024

Introduction

Adrenal cortical carcinoma (ACC), is a rare endocrine adrenal malignancy that occurs in 0.7-2 cases per million per year.1,2 The biological behavior of tumors in patients with ACC is usually aggressive, and 50%-70% of these patients have hormone overdose symptoms and signs. Glucocorticoids and/or androgens are hypersecreted by two-thirds of patients.3,4 Excess hormone secretion in patients with ACC causes increased abdominal mass, weight loss, and other constitutional symptoms.4 Females

‘Clinical laboratory, Shenzhen Longhua District Central Hospital, Shenzhen, China

2Department of Gynecology, Shenzhen Longhua District Central Hospital, Shenzhen, China

3Department of Parasitology, Guangdong Medical University, Zhanjiang, China

Corresponding author:

Yongli Situ, Department of Parasitology, Guangdong Medical University, No. 2 East Wenming Road, Zhanjiang, 524023, China. Email: styl1987@126.com

are more likely to suffer from the disease than males (1.5:1).5,6 Due to the difficult clinical presentation of ACC and the fact that most patients are diagnosed at a late metastatic stage, the prognosis for ACC is extremely poor. Patients with locoregional ACC are treated surgically; however, approximately 75% experience recurrence after surgery.7 Furthermore, for patients with advanced meta- static disease, overall survival is 12-15 months and 15% at 5 years.8,9 Compared to patients with other tumor types, patients with ACC do not benefit from improvements in overall tumor treatment. Chemotherapy and radiation therapy are ineffective in most ACC cases. Mitotane is the only drug approved by the US Food and Drug Administration for ACC and is usually only temporarily effective and has obvious side effects.10,11 Complete tumor resection remains the only curative treatment.12 Therefore, it is important to identify the target genes and regulatory targets that affect the prognosis of patients with ACC under circumstances of limited therapeutic drugs, poor therapeutic effects, and a high risk of disease recurrence.

Apolipoprotein C1 (APOC1) and Apoprotein E (APOE) are members of the apolipoprotein family. APOC1, the smallest apolipoprotein (6.6 kDa), is a triglyceride-rich and high-density lipoprotein closely related to inflamma- tion, immunity, sepsis, and diabetes.13 APOE, a 34 glyco- protein, is a high-density lipoprotein with antioxidant, anti-inflammatory, and antiatherogenic properties.14 Recently, several studies have shown that APOC1 and APOE are involved in the development and occurrence of multiple cancers. APOC1 and APOE are diagnostic and prognostic biomarkers for many cancers. Interestingly, APOC1 and APOE expression levels were different in dif- ferent tumors. Therefore, the function of APOC1 and APOE in different tumors may differ. For example, APOC1 is significantly overexpressed and its overexpres- sion is associated with lymph node metastasis, tumor-node-metastasis stage, distant metastasis, and poor prognosis in colorectal cancer, gastric cancer, and clear cell renal cell carcinoma.15-17 The overexpression level of APOE in endometrial cancer has been correlated with histo- logical grade, lymph node metastasis, and the International Federation of Gynecology and Obstetrics stage.18 However, APOC1 has significantly lower expression in esophageal cancer and hepatocellular carcinoma, which is associated with reshaping the tumor immune microenvironment and inhibiting the proliferation, migration, and invasion of cancer cells.19,20 Serum APOE levels tend to decrease in patients with laryngeal squamous cell carcinoma.21 It is cur- rently unknown if APOC1 and APOE play a role in ACC. Hence, in this study, the expression of APOC1 and APOE in patients with ACC, as well as the gene regulatory network, prognostic value, target prediction, and potential therapeutic agents were examined. These data clarify the relationships between APOC1, APOE, and ACC.

Materials and methods

Gene expression profiling interactive analysis (GEPIA)

GEPIA (http://gepia.cancer-pku.cn/index.html) is an intuitive network application tool to assess the biological relationships between gene expression and prognostic information in patients with cancer.22 GEPIA was used to analyze the rela- tionships between gene expression, tumor pathological stages, and prognosis. The screening criteria were: (1) genes: APOC1 and APOE; (2) dataset: ACC; and (3) 77 patients with ACC; threshold setting conditions: P-value cutoff ≤ 0.05. Student’s t-test was used to analyze the expres- sion of APOC1 and APOE in ACC. Kaplan-Meier curves were used to analyze the prognosis of patients with ACC.23

The university of Alabama at Birmingham CANcer data analysis portal (UALCAN)

UALCAN (http://ualcan.path.uab.edu/analysis.html) facilitates tumor subgroup gene expression and survival analyses.22 UALCAN was used to analyze APOC1 and APOE expression in ACC. The “Expression Analysis” module of the UALCAN database was used to analyze The Cancer Genome Atlas (TCGA) gene expression data. The screening criteria were set as: (1) genes: APOC1 and APOE; (2) dataset: ACC; (3) 79 patients with ACC (31 male and 48 female); threshold setting conditions: p-value cutoff=0.05. Student’s t-test was used for comparative analyses.23

Biomarker exploration of solid tumors (BEST)

BEST (https://rookieutopia.com/app_direct/BEST/) pro- vides a curated database and innovative analytical pipelines to explore cancer biomarkers at high resolution. Protein expression, immune cell infiltration, candidate agents, and immunotherapy of APOC1 and APOE in ACC were ana- lyzed using the BEST software. The “Cell infiltration,” “Immunotherapy,” and “Candidate agents” modules of the BEST database were used to analyze Gene Expression Omnibus and TCGA gene expression data. The screening criteria were set as: (1) genes: APOC1 and APOE; and (2) dataset: ACC (10 datasets with 508 patients).23

Bioportal for cancer genomics (cBioPortal)

cBioPortal (http://cbioportal.org) is an online database used for tumor gene mutation analysis. cBioPortal was used to analyze gene alterations to APOC1, APOE, and the top 50 altered neighboring genes (ANGs), respectively.22 A total of 75 ACC samples were analyzed and mRNA expression Z-scores were obtained relative to all samples (log RNA Seq V2 RSEM) using a z-score threshold of +2.0.23

Search tool for the retrieval of interacting genes/ proteins (STRING) and gene multiple association network integration algorithm (GeneMANIA)

STRING (https://string-db.org/cgi/input.pl) and GeneMANIA (http://www.genemania.org) are online databases used to analyze gene-protein and protein- protein interactions.22 STRING was used to build a low- confidence level (0.150) protein-protein interaction (PPI) network to screen criteria for species defined as humans. Cytoscape software (version 3.10.2, cytoHubba plug-ins) was used to discover the core pro- teins in PPI network. GeneMANIA was used to explore the function of APOC1, APOE, and the top 50 ANGs, respectively.23

Metascape

Metascape (https://metascape.org) is an online database used to analyze the functions and signaling pathways of genes and proteins.22 In this study, Metascape was used to analyze the function and signaling pathways of APOC1, APOE, and the top 50 ANGs, respectively.23

Transcriptional regulatory relationships unraveled by sentence-based text-mining (TRRUST)

TRRUST (https://www.grnpedia.org/trrust/) is an online database used to analyze regulatory targets of gene tran- scription.22 Here, TRRUST was used to identify the key transcriptional regulators of APOC1, APOE, and the top 50 ANGs, respectively.23

Linkedomics

LinkedOmics (http://www.linkedomics.org/) is a public online platform used to analyze correlations between differ- entially expressed genes related to tumor target genes and to predict microRNA (miRNA) and kinase targets.22 LinkedOmics was used to identify kinase and miRNA targets, as well as differentially expressed genes related to APOC1 and APOE, respectively.23

Genomics of drug sensitivity in cancer analysis

The Genomics of Drug Sensitivity in Cancer database (http://www.cancerRxgene.org) is a specialized public data- base to obtain information on potential anticancer drugs.23 This database was used to identify drugs targeting APOC1 and APOE and to predict their anti-ACC activity.

Results

APOC1 and APOE expression, prognosis, and genetic alteration in ACC

As shown in Figure 1(a) to (i), APOC1 and APOE are sig- nificantly downregulated in patients with ACC (p<0.05). A significant correlation between APOC1 expression and the pathological stage was observed in patients with ACC (p<0.05) (Figure 1(j) and (k)). However, APOC1 and APOE expression were lower in male patients than that in female patients with ACC (p < 0.05) (Figure 1(l) and (m)). Patients with ACC who expressed low levels of APOC1 had longer overall survival times (p = 0.0083) (Figure 1(n)). Patients with ACC with low APOC1 and APOE expression had longer disease-free survival times than those with high APOC1 and APOE expression levels (p=0.017 and p=0.049, respectively) (Figure 1(o) and (q)). According to these results, APOC1 and APOE expression levels were altered by 9% and 15%, respectively, in patients with ACC (Figure 1(r) and (s)).

Interaction network of APOC1, APOE, and ANGs in ACC

As shown in Tables 1 and 2, APOC1 and APOE ANG alter- ation frequencies of ≥ 28.57% and ≥ 27.27% were observed in the 50 most frequent ANGs in patients with ACC, respectively. Ring finger protein 135 (42.86%), adhesion G protein-coupled receptor A2 (28.57%), and adhesion G protein-coupled receptor G2 (28.57%) were the most fre- quent ANGs of APOC1 in patients with ACC (Table 1). Furthermore, the most frequent ANGs of APOE in patients with ACC were mucin 2 (MUC2) (72.73%), MUC4 (63.64%), and outer dense fiber of sperm tails 3 like 2 (36.36%) (Table 2). A total of 38 nodes and 150 edges were identified in the PPI network (Figure 2(a)). On the basis of the PPI network, the core proteins were extracted with Cytoscape software. The results showed the core protein with high scoring were as follows (Top 5): TOMM40, PVRL2, CLPTM1, APOC1, and APOC2 (Figure 2(e)). A complex interaction network was discov- ered between APOC1 and its ANGs by co-expression, co-localization, physical interactions, shared protein domains, and genetic interactions (Figure 2(b)). Moreover, 43 nodes and 152 edges were obtained in the PPI network (Figure 2(c)). Our results showed CDC34, ODF3L2, C2CD4C, MIER2, and TPGS1 were the core protein with high scoring (Top 5) (Figure 2((f)). An intricate network of interactions existed between APOE and its ANGs, including co-expression, physical interactions, path- ways, shared protein domains, and co-localization (Figure 2(d)).

Figure 1. The transcription levels, prognostic value, and genetic alteration of APOC1 and APOE in ACC (GEPIA, BEST, UALCAN, and cBioPortal). (a) Boxplot showing transcription level of APOC1 in patients with ACC (GEPIA); (b) Boxplot showing transcription level of APOE in patients with ACC (GEPIA); (c-g) Boxplot showing transcription level of APOC1 in patients with ACC (BEST); (h and i) Boxplot showing transcription level of APOE in patients with ACC (BEST); (j and k) Boxplot showing correlation between the pathological stage and different expressed APOC1 in patients with ACC (BEST); (l) Boxplot showing transcription level of APOC1 in patients with ACC based on the sex (UALCAN); (m) Boxplot showing transcription level of APOE in patients with ACC based on sex (UALCAN); (n) The overall survival curve of APOC1 in patients with ACC (GEPIA); (o) The disease-free survival curve of APOC1 in patients with ACC (GEPIA); (p) The overall survival curve of APOE in patients with ACC (GEPIA); (q) The disease-free survival curve of APOE in patients with ACC (GEPIA); (r) Genetic alteration of APOC1 in patients with ACC (cBioPortal); (s) Genetic alteration of APOE in patients with ACC (cBioPortal); * p < 0.05.

(a)

(b)

(c)

(d)

e

(f)

GSE10927

GSE12368

GSE143383

GSE33371

(g)

GSE90713

=

APOCI

*

2

APOE

Wilcoxon, p = 0.00041

Wilcoxon, p = 0.022

T-test, p = 1.9e-13

Wilcoxon, p = 0.00041

2

T-test, p = 1.2e-12

~

1

1

1

3 ”

1

APOC1 Expression (z-score)

·

APOC1 Expression (z-score)

.

APOC1 Expression (z-score)

1

APOC1 Expression (z-score)

APOC1 Expression (z-score)

.

:

E

.

8

1

y

10

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6

21

Q

0

0

0

0

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1

8

1

1

… .

+

-1

·

-1

·

00

2

APOCI

APOC1

2

APOCI

2

APOCI

·!

-2

APOC1

0

0

Normal

Tumor

Normal

Tumor

Normal

Tumor

Normal

Tumor

Normal

Turnor

(num(T)-77; num(N)=128)

ACC

(num[T]=77; num(N)=128)

ACC

Tissue

Tissue

Tissue

Tissue

Tissue

(h)

(i)

(j)

(k)

GSE143383

GSE90713

GSE76019

GSE76021

(1)

(m)

Wilcoxon, p = 0.04

Wilcoxon, p = 0.022

2

ruskal-Wallis, p = 0.044

Anova, p = 0.015

N

1

·

Expression of APOC1 in ACC based on patient’s gender

Expression of APOE in ACC based on patient’s gender

1

2

3500

APOE Expression (z-score)

APOE Expression (z-score)

APOC1 Expression (z-score)

APOC1 Expression (z-score)

12.5k -

3000

.

.

1

0

0

0

10k

Transcript per million

2500

Transcript per million

2000

7.5k

1

0

1

1500

5k

·

2

1000

-2

·

-1

500-

2.5k

2

APOE

APOE

.

0

0

Normal

Tumor

Normal

Tumor

APOC1

APOCI

Female

Made

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·

-

+

TOGA samples

.

-

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TOGA samples

$

*

Tissue

Tissue

Stage

Stage

(n)

(p)

(q)

Overall Survival

(0)

Disease Free Survival

Overall Survival

Disease Free Survival

:

Low TPM

High APOC1 IPM

:

Low APOC1 TPM

2

Low APOE TPM

9

Logrank p=0.0083

High

Logrank p=0.017

High APOE TPM Logrank p=0.058

LOW APOE TPM

High APOE TPM

HR[high)=2.9

Logrank p=0.049

3

p(HR)-0.012

g

HR(high)-2.3

n[high)=38

p(HR)=0.02

8

HR(high)=2.1

p(HR)=0.063

g

-HR(high)=2

4

ngon)-38

n[high]=38

p(HR)-0.054

Percent survival

Percent survival

MỘT -38

Percent survival

m(high)=38 now)=38

Percent survival

n(high0-38

OK

0.6

8

0.4

0.4

3

2

3

3

3

8

APOCI

8

APOCI

8

APOE

8

APOE

0

50

100

150

0

50

100

150

0

50

100

150

0

50

100

150

Months

Months

Months

Months

(r)

Altered in 7 (9%) of 75 patients

APOC1

9%

Genetic alteration

Amplification

mRNA High

mRNA Low

No alterations

(s)

Altered in 11 (15%) of 75 patients

APOE

15%

Genetic alteration

Missense Mutation (unknown significance)

Amplification

mRNA Low

No alterations

Gene ontology function and Kyoto encyclopedia of genes and genomes (KEGG) pathway enrichment analysis of APOC1, APOE, and ANGs in ACC

As shown in Figure 3(a), molecular functions related to APOC1 and its ANGs are mainly associated with protein tyrosine kinase binding, phosphoric ester hydrolase activ- ity, DNA and transcription factor binding, and channel activity. Moreover, organophosphate ester transport, rRNA processing, cell surface receptor signaling pathways

involved in cell-cell signaling, negative regulation of phos- phate metabolic processes, and positive regulation of cyto- kine production were the main biological processes of APOC1 and its ANGs (Figure 3(b)). Cellular components of APOC1 and its ANGs included very-low-density lipo- protein particles, actin-based cell projections, and apical plasma membranes (Figure 3(c)). However, the molecular functions of APOE and its ANGs included protein tyrosine kinase binding, protein domain-specific binding, ubiquitin- protein transferase activity, and transcriptional co-regulator

Table 1. The top 50 ANG of APOC1 in ACC (cBioPortal). Table 2. The top 50 ANG of APOE in ACC (cBioPortal).
GeneAltered groupUnaltered groupp-ValueGeneAltered groupUnaltered groupp-Value
RNF1353 (42.86%)1 (1.47%)0.00199MUC28 (72.73%)14 (21.88%)0.00168
ADGRA22 (28.57%)0 (0.00%)0.00757MUC47 (63.64%)13 (20.31%)0.00613
ADGRG22 (28.57%)0 (0.00%)0.00757ODF3L24 (36.36%)2 (3.13%)0.00345
APOC1P12 (28.57%)0 (0.00%)0.00757PARP84 (36.36%)2 (3.13%)0.00345
APOC22 (28.57%)0 (0.00%)0.00757BTNL94 (36.36%)3 (4.69%)0.00741
APOC42 (28.57%)0 (0.00%)0.00757MROH2B4 (36.36%)3 (4.69%)0.00741
BCAM2 (28.57%)0 (0.00%)0.00757ATXN7L33 (27.27%)0 (0.00%)0.00244
CACNA1D2 (28.57%)0 (0.00%)0.00757AXL3 (27.27%)0 (0.00%)0.00244
CCDC542 (28.57%)0 (0.00%)0.00757C2CD4C3 (27.27%)0 (0.00%)0.00244
CEACAM162 (28.57%)0 (0.00%)0.00757C8ORF173 (27.27%)0 (0.00%)0.00244
CEACAM192 (28.57%)0 (0.00%)0.00757CAVIN13 (27.27%)0 (0.00%)0.00244
CEACAM202 (28.57%)0 (0.00%)0.00757CBLC3 (27.27%)0 (0.00%)0.00244
CEACAM22P2 (28.57%)0 (0.00%)0.00757CD300LG3 (27.27%)0 (0.00%)0.00244
CENPF2 (28.57%)0 (0.00%)0.00757CDC343 (27.27%)0 (0.00%)0.00244
CLPTM12 (28.57%)0 (0.00%)0.00757CFAP97D13 (27.27%)0 (0.00%)0.00244
GPA332 (28.57%)0 (0.00%)0.00757CNTD13 (27.27%)0 (0.00%)0.00244
IGSF232 (28.57%)0 (0.00%)0.00757DUSP33 (27.27%)0 (0.00%)0.00244
NECTIN22 (28.57%)0 (0.00%)0.00757ETV43 (27.27%)0 (0.00%)0.00244
RN7SL48P2 (28.57%)0 (0.00%)0.00757FAM138F3 (27.27%)0 (0.00%)0.00244
SCML22 (28.57%)0 (0.00%)0.00757FAM215A3 (27.27%)0 (0.00%)0.00244
TMPRSS22 (28.57%)0 (0.00%)0.00757GJD33 (27.27%)0 (0.00%)0.00244
TOMM402 (28.57%)0 (0.00%)0.00757GZMM3 (27.27%)0 (0.00%)0.00244
ZC3H132 (28.57%)0 (0.00%)0.00757IRGQ3 (27.27%)0 (0.00%)0.00244
ZNF1802 (28.57%)0 (0.00%)0.00757MEGF83 (27.27%)0 (0.00%)0.00244
ZNF5162 (28.57%)0 (0.00%)0.00757MEOX13 (27.27%)0 (0.00%)0.00244
ARL5C2 (28.57%)1 (1.47%)0.0217MIER23 (27.27%)0 (0.00%)0.00244
BCL32 (28.57%)1 (1.47%)0.0217MPP23 (27.27%)0 (0.00%)0.00244
C19ORF122 (28.57%)1 (1.47%)0.0217OR4F173 (27.27%)0 (0.00%)0.00244
CBLC2 (28.57%)1 (1.47%)0.0217PCGF23 (27.27%)0 (0.00%)0.00244
CCNE12 (28.57%)1 (1.47%)0.0217PIP4K2B3 (27.27%)0 (0.00%)0.00244
CD300LG2 (28.57%)1 (1.47%)0.0217PPY3 (27.27%)0 (0.00%)0.00244
CFAP97D12 (28.57%)1 (1.47%)0.0217PSMB33 (27.27%)0 (0.00%)0.00244
CLCN72 (28.57%)1 (1.47%)0.0217PYY3 (27.27%)0 (0.00%)0.00244
DDX5 12 (28.57%)(1.47%)0.0217RNA5SP4623 (27.27%)0 (0.00%)0.00244
DUSP32 (28.57%)1 (1.47%)0.0217SOST3 (27.27%)0 (0.00%)0.00244
ENPP22 (28.57%)1 (1.47%)0.0217STK113 (27.27%)0 (0.00%)0.00244
ETV42 (28.57%)1 (1.47%)0.0217THEG3 (27.27%)0 (0.00%)0.00244
FAM215A2 (28.57%)1 (1.47%)0.0217TPGS13 (27.27%)0 (0.00%)0.00244
G6PC32 (28.57%)1 (1.47%)0.0217WASH5P3 (27.27%)0 (0.00%)0.00244
GALNT92 (28.57%)1 (1.47%)0.0217AHDC13 (27.27%)1 (1.56%)0.00896
GSTT22 (28.57%)1 (1.47%)0.0217C63 (27.27%)1 (1.56%)0.00896
KRT262 (28.57%)1 (1.47%)0.0217C73 (27.27%)1 (1.56%)0.00896
MEOX12 (28.57%)1 (1.47%)0.0217CARD63 (27.27%)1 (1.56%)0.00896
MPP22 (28.57%)1 (1.47%)0.0217HERC23 (27.27%)1 (1.56%)0.00896
NLRP112 (28.57%)1 (1.47%)0.0217JUP3 (27.27%)(1.56%)0.00896
NOC4L2 (28.57%)1 (1.47%)0.0217KRT123 (27.27%)(1.56%)0.00896
NOL112 (28.57%)1 (1.47%)0.0217KRT323 (27.27%)(1.56%)0.00896
ODAD42 (28.57%)1 (1.47%)0.0217MPP33 (27.27%)(1.56%)0.00896
PHKA22 (28.57%)1 (1.47%)0.0217NLRP83 (27.27%)1 (1.56%)0.00896
PPEF12 (28.57%)1 (1.47%)0.0217NNT3 (27.27%)1 (1.56%)0.00896

activity (Figure 3(d)). Furthermore, the main biological pro- cesses of APOE and its ANGs were cytolysis, negative regulation of kinase activity, regulation of the canonical Wnt signaling pathway, response to reactive oxygen species, protein ubiquitination, response to growth factors, cell surface receptor signaling pathways involved in

cell-cell signaling, and histone modification (Figure 3(e)). The cellular components of APOE and its ANGs included intermediate filaments, cell-cell junctions, and the extracel- lular matrix (Figure 3(f)). KEGG pathway analysis of APOE and its ANGs revealed ubiquitin-mediated proteoly- sis and regulation of the actin cytoskeleton (Figure 3(g)).

Figure 2. Interaction analyses of APOC1, APOE, and their ANG in ACC (STRING and GeneMANIA). (a) PPI network of APOC1 and its ANG in patients with ACC (STRING); (b) Network analyses of APOC1 and its ANG in patients with ACC (GeneMANIA); (c) PPI network of APOE and its ANG in patients with ACC (STRING); (d) Network analyses of APOE and its ANG in patients with ACC (GeneMANIA); (e) Core proteins in PPI network of APOC1 and its ANG in patients with ACC (Cytoscape); (f) Core proteins in PPI network of APOE and its ANG in patients with ACC (Cytoscape).

CONELG

(a)

3

EMPRE

GIMAIS

(b)

GPRS4

GP#124

NURPHÍ

GSTTAR

PPEFI

NOLIE

casotiz

DUSPE

DOXSL

CONEL

MÉGIS

CENPE

NOCAL

De3H13

CLIMA

PONAD

CACHALO

CEACANDID

APOCA

CEACAMIE

APOČ!

Networks

TMPRSSZ

Co-expression

CEACAMIS

Co-localization

PVR12

APOCZ

CLIENT

Physical Interactions

CBLG

Shared protein domains

Genetic Interactions

SCAM

APOC1

APOC1

(c)

(d)

NLRP6

-

MUCH

CARDS

GALNIT32

VIROFOR

ETW

sost

STKIS

MƯỜNG

PYY

APOIE

RGQ

ODFILZ

TPGS1

ppy

CHLC

HERCE

NNIT

DUSPI

MIERCE

THEG

KIRT12

UPP3

OR4FIT

UPPZ

CNTDA

Psund

GZMM

Networks

KAT32

COMDALG

GJ00

Co-expression

C17ort1.05

MEGFÉ

PCOFZ

Physical Interactions

83M.

Pathway

Shared protein domains

APOE

Co-localization

APOE

(e)

(f)

CLCN7

VEOXI

DDX51

OSTT 28

NOL11

CEACAM

JALNTIE

PERF

PYY

BCAM

NLRP11

HORCZ

JUP

ETV4

GPA33

MUCA

KORT32

THEG

GPR124

PPEF1

8

ETW

2

C19omf12

PVRL2

APOC1

CEACAM16

POOP2

2

5523

2

MPP2

OUSP3

APOE

MERZ

COCM

NNT

2

NOCAL

EACAM2

STK11

PSMBS

B

5

S

APOCZ

OMMA

DTNLS

DUSP

CONE1

ENPP2

25

DOPSLI

CARDS

APOCA

APTM

PHKA2

3

CNTDA

2

SOST

TPG41

CRL.C

ZC3H13

ARLSC

PPY

ALAPB

CBLC

KRT12

GAGNAIT

BCLJ

MPP2

SCML2

GPR64

CENPF

IMPRSS

GSF23

RGO

MPP3

MEGF

AIL

APOC1

APOE

Transcription factor, miRNA, and kinase targets of APOC1 and APOE in patients with ACC

As shown in Table 3, E2F1 is the key transcription factor involved in the network of APOC1 and its ANGs (p< 0.05). E2F1 regulates the functions of cyclin E1 and ETS variant transcription factor 4 (ETV4). Moreover, E2F1, signal transducer and activator of transcription 3

(STAT3), specificity protein 1 (SP1), and tumor protein p53 (TP53) were key transcription factors for APOE and its ANGs (p<0.05). Among these, ETV4 and MUC4 were the main regulatory genes of E2F1. STAT3 regulated MUC4 and serine/threonine kinase 11(STK11) function and APOE, MUC4, and STK11 were the main regulatory genes of SP1. TP53 regulated coactivator associated arginine

Figure 3. Go function and KEGG pathways enrichment analyses of APOC1, APOE, and their ANG in patients with ACC (metascape). (a) Molecular functions of APOC1 and its ANG in patients with ACC; (b) Biological processes of APOC1 and its ANG in patients with ACC; (c) Cellular components of APOC1 and its ANG in patients with ACC; (d) Molecular functions of APOE and its ANG in patients with ACC; (e) Biological processes of APOE and its ANG in patients with ACC; (f) Cellular components of APOE and its ANG in patients with ACC; (g) KEGG pathway analysis of APOE and its ANG in patients with ACC. The GO enriched terms are colored by p-value, where terms containing more genes tend to have more significant p-value.

(a)

Molecular functions (APOC1)

(b)

Biological processes (APOC1)

GO:1990782: protein tyrosine kinase binding

GO:0015748: organophosphate ester transport

GO:0042578: phosphoric ester hydrolase activity

GO:0006364: rRNA processing

GO:0140297: DNA-binding transcription factor binding

GO:0044782: cilium organization

GO:0015267: channel activity

GO: 1905114: cell surface receptor signaling pathway involved in cell-cell signaling

GO:0033365: protein localization to organelle

0.0

0.5

1.0

1.5

2.0

2.5

3.0

GO:0045936: negative regulation of phosphate metabolic process

-log10(P)

GO:0001819: positive regulation of cytokine production

0.0

0.5

1.0

1.5

2.0

-log10(P)

2.5

(e)

Cellular components (APOC1)

(d)

Molecular functions (APOE)

GO:0034361: very-low-density lipoprotein particle

GO:1990782: protein tyrosine kinase binding

GO:0098858: actin-based cell projection

GO:0019904: protein domain specific binding

GO:0016324: apical plasma membrane

GO:0004842: ubiquitin-protein transferase activity

GO:0003712: transcription coregulator activity

0

1

2

3

4

5

-log10[P)

0.0

0.5

1.0

1.5

2.0

2.5

3.0

-log10(P)

(f)

Biological processes (APOE)

(f)

GO:0007283: spermatogenesis

Cellular components (APOE)

GO:0019835: cytolysis

GO:0033673: negative regulation of kinase activity

GO:0005882: intermediate filament

GO:0060828: regulation of canonical Wnt signaling pathway

GO:0005911: cell-cell junction

GO:0000302: response to reactive oxygen species

GO:0016567: protein ubiquitination

GO:0031012: extracellular matrix

GO:0001894: tissue homeostasis

GO:0070848: response to growth factor

0.0

0.5

1.0

1.5

2.0

GO:0007610: behavior

-log10[P)

GO:0043009: chordate embryonic development

GO:1905114: cell surface receptor signaling pathway involved in cell-cell signaling GO:0016570: histone modification

GO:0032940: secretion by cell

0

1

2

3

4

5

-log10[P)

(g)

KEGG pathway (APOE)

hsa04120: Ubiquitin mediated proteolysis

hsa04810: Regulation of actin cytoskeleton

0.0

0.5

1.0

1.5

2.0

2.5

-log10(P)

Table 3. Key regulated factor of APOC1, APOE, and their top 50 ANG in ACC (TRRUST).
GeneKey TFDescriptionRegulated geneP-value
APOC1E2F1E2F transcription factor 1CCNE1, ETV40.0374
APOEE2F1E2F transcription factor 1ETV4, MUC40.039
APOESTAT3signal transducer and activator of transcription 3 (acute-phase response factor)MUC4, STK110.0433
APOESP1Sp1 transcription factorAPOE, MUC4, STK110.0472
APOETP53tumor protein p53CARM1, DNMT10.0451

methyltransferase 1 and DNA methyltransferase 1 function. miRNA targets of APOC1 and APOE were identified using LinkedOmics software (Table 4). MiR-19A, miR-19B, miR-182, and miR-302C were the miRNA targets of APOC1 in ACC (p<0.001) (Table 4). However, the miRNA targets of APOE in ACC were miR-19A, miR-19B, miR-181A, miR-181B, miR-181C, miR-181D, and miR-493 (p<0.001) (Table 4). Erb-B2 receptor tyro- sine kinase 2 (ERBB2), transforming growth factor beta receptor 1 (TGFBR1), and cyclin dependent kinase 1 (CDK1) were kinase targets of APOC1 in patients with ACC (p<0.01) (Table 5). Moreover, the kinase targets of APOE were mitogen-activated protein kinase kinase 7 (MAP2K7), CDK1, and CDK2 in patients with ACC (p<0.01) (Table 5).

Correlation of differentially expressed genes and expression of APOC1 and APOE in patients with ACC

As shown in Figure 4(a) and (d), 4471 and 4021 genes are closely related to APOC1 and APOE, respectively, in patients with ACC. Among them, 2115 and 1696 genes showed positive correlations whereas 2356 and 2325 genes showed negative correlations with APOC1 and APOE expression, respectively (Figure 4(a) and (d)). In addition, 50 genes were significantly positively and nega- tively correlated with APOC1 and APOE expression in patients with ACC (Figure 4(b), (c), (e), and (f)). Among them, APOC1 expression was strongly positively associated with APOE (Pearson correlation coefficient (PCC) =

Table 4. The top three miRNA target of APOC1 and APOE in ACC (linkedOmics).
GeneGene setLeading edge numberP-valueFDR
APOC1TTTGCAC,162<2.2e-16<2.2e-16
miR-19A,
miR-19B
TAGAACC,15<2.2e-16<2.2e-16
miR-182
ATGTTAA,84<2.2e-16<2.2e-16
miR-302C
APOETTTGCAC,200<2.2e-16<2.2e-16
miR-19A,
miR-19B
TGAATGT,161<2.2e-16<2.2e-16
miR-181A,
miR-181B,
miR-181C,
miR-181D
ATGTACA,117<2.2e-16<2.2e-16
miR-493

0.7713, p=9.06e-17) (Figure 4(g)), apolipoprotein C1 pseudogene 1 (APOC1P1) (PCC=0.6812, p=4.872e-12) (Figure 4(h)), and negatively associated with sparc/osteo- nectin, cwcv and kazal-like domains proteoglycan (testican) 1 (SPOCK1) (PCC =- 0.5938, p= 8.008e-9) (Figure 4(i)). APOE expression was positively associated with APOC1 (PCC=0.7713, p=9.06e-17) (Figure 4(j)), family with sequence similarity 196 member B (FAM196B) (PCC= -0.5897, p=1.076e-8) (Figure 4(k)), and calcium/cal- modulin dependent protein kinase 1D (CAMK1D) (PCC = -0.5669, p=5.122e-8) (Figure 4(1)) expression.

Correlation of immune cell infiltration and APOC1 and APOE expression and anti-programmed cell death protein 1 (PD1)/programmed death-ligand 1 (pd-L1) immunotherapy in ACC

As shown in Figure 5(a) to (h), APOC1 and APOE expres- sion in patients with ACC is positively associated with immune cell infiltration (p<0.05). In addition, APOC1 was significantly downregulated in patients with ACC treated with anti-PD1 (p=0.01) (Figure 5(i)). However, APOE expression in patients with ACC treated with anti-PD1/PD-L1 was significantly upregulated (p=0.05) (Figure 5(j)).

Therapeutic drugs of APOC1 and APOE in ACC

The BEST database was used to evaluate APOC1 and APOE expression, where high expression indicated drug

resistance; pilaralisib and elesclomol were identified as first-line drugs, respectively (Figure 6(a) and (f)). Next, the Genomics of Drug Sensitivity in Cancer database was used to evaluate the inhibitory effects of pilaralisib and ele- sclomol on ACC cell lines. Pilaralisib inhibited 919 cell lines with area under the curve (AUC) values greater than 0.374 (Figure 6(c)). On these cell lines, pilaralisib had a good inhibitory effect (0.573≤IC50 (uM) ≤740) (Figure 6(b)). Moreover, pilaralisib strongly inhibited the ACC cell line SW13 (AUC values =0.857985, IC50 (µM)= 16.488967) (Figure 6(d) and (e)). In contrast, ele- sclomol inhibited 921 cell lines, with AUC values greater than 0.0209 (Figure 6(h)). On these cell lines, elesclomol had a good inhibitory effect (0.000231 ≤IC50 (UM)≤ 10.3) (Figure 6(g)). Furthermore, elesclomol also strongly inhibited SW13 (AUC values =0.421688, IC50 (M)= 0.007628) (Figure 6(i) and (j)).

Discussion

Abnormal APOC1 and APOE expression have been observed in various tumors. However, APOC1 and APOE expression in patients with ACC remains unknown. Strong downregulation of APOC1 and APOE expression has been observed in patients with ACC. APOC1 and APOE expression levels were lower in male patients with ACC than those in female patients with ACC. Low APOC1 and APOE expression levels were associated with longer survival times in patients with ACC. The number of cases in the female population generally outnumbers that of the male population (1.5:1),5,6 suggesting that sex differences in APOC1 and APOE expression may be an important factor affecting the prognosis of patients with ACC. However, this hypothesis requires further investiga- tion. These findings suggest that APOC1 and APOE are potential targets for ACC therapy. Next, the abnormal expression of APOC1 and APOE in patients with ACC was explained through genetic alterations. These results revealed that APOC1 and APOE expression were altered by 9% and 15%, respectively, in patients with ACC, with the type of genetic alteration mainly including amplification and high and low RNA levels. In addition, genetic altera- tions may lead to decreased expression of APOC1 and APOE. However, further investigation is required to address this issue. Finally, the prognostic value of APOC1 and APOE expression in patients with ACC was evaluated. According to these results, patients with ACC with low APOC1 and APOE expression had longer survival times than those with high APOC1 and APOE expression. Thus, APOC1 and APOE may serve as potential prognostic markers for patients with ACC.

The potential interactions between APOC1, APOE, and the ANGs were evaluated. As a result of co-expression, co-localization, and shared protein domains, APOC1, APOE, and the ANGs were linked to a complex interaction

Table 5. The top three kinase target of APOC1 and APOE in ACC (linkedOmics).
GeneKinase targetDescriptionLeading edge numberp-value
APOC1Kinase_ERBB2erb-b2 receptor tyrosine kinase 260.0036900
Kinase_TGFBR1transforming growth factor beta receptor 160.0045662
Kinase_CDK1cyclin dependent kinase 1600.0068729
APOEKinase_MAP2K7mitogen-activated protein kinase kinase 73<2.2e-16
Kinase_CDK1cyclin dependent kinase 157<2.2e-16
Kinase_CDK2cyclin dependent kinase 2770.0024752

network. The functions of APOC1, APOE, and the ANGs were also evaluated. The molecular functions related to APOC1 and its ANGs mainly included protein tyrosine kinase binding, phosphoric ester hydrolase activity, DNA and transcription factor binding, and channel activity. Protein tyrosine kinases play important roles in various cel- lular processes, including growth, motility, differentiation, and metabolism. Abnormal expression of protein tyrosine kinases usually leads to cell proliferation disorders and is closely related to tumor invasion, metastasis, acquired resistance, and tumor angiogenesis.24 DNA- and RNA-binding proteins are a broad class of molecules that regulate numerous cellular processes in all living organ- isms, and create intricate dynamic multilevel networks to control nucleotide metabolism and gene expression. These interactions are highly regulated, and their dysregulation contributes to the development of various diseases, includ- ing cancer.25 Targeting transcription factors and DNA-binding proteins is an important strategy in cancer treatment.26,27 The molecular functions of APOE and its ANGs included protein tyrosine kinase binding, ubiquitin- protein transferase activity, and transcriptional co-regulatory activity. Ubiquitination, one of the most important post-translational modifications, plays a versatile role in cancer-related pathways and is involved in protein metabolism, cell cycle progression, apoptosis, and tran- scription.28 The regulation of ubiquitination is an important cancer treatment strategy. The current results showed that the biological processes related to APOC1 and its ANGs mainly included rRNA processing, negative regulation of phosphate metabolic processes, and positive regulation of cytokine production. The biological processes affecting tumor proliferation, invasion, metastasis, and metabolism have been reported.29-31 The biological processes related to APOE and its ANGs mainly included cytolysis, negative regulation of kinase activity, protein ubiquitination, response to growth factors, and histone modification. These biological processes affect tumor proliferation, inva- sion, metastasis, and angiogenesis.32-34 KEGG pathway analysis of APOE and its ANGs showed that ubiquitin- mediated proteolysis and actin cytoskeleton regulation were enriched. Actin filaments are major components of the cytoskeleton in eukaryotic cells. Mutations and abnor- mal expression of cytoskeletal and cytoskeleton-associated proteins play important roles in cancer cell resistance to

chemotherapy and metastasis.35 Taken together, APOC1, APOE, and the ANGs may contribute to the development and progression of ACC. Thus, ACC might be treated by regulating these genes and signaling pathways.

Transcription factors and miRNA and kinase targets of APOC1 and APOE were examined in patients with ACC. These results showed that E2F1, STAT3, SP1, and TP53 were key transcription factors of APOC1, APOE, and the ANGs in patients with ACC. E2F1 cooperates with enhan- cer of zeste 2 polycomb repressive complex 2 subunit to stimulate the expression of genes involved in ACC aggres- siveness.36 STAT3 promotes angiogenesis in patients with ACC, thereby making STAT3 a selective target for molecular-targeted therapy of ACC.37 SP1 is a well-known member of the transcription factor family that plays import- ant roles in cell growth, differentiation, apoptosis, and car- cinogenesis.38 However, its role in ACC has not yet been reported. ACC is a rare tumor type associated with TP53 mutations. Genetic susceptibility caused by mutations in TP53 is associated with approximately 50% of childhood ACC cases, but only 3%-6% of adult ACC cases.39 Moreover, miR-19A, miR-19B, miR-182, miR-302C, miR-181A, miR-181B, miR-181C, and miR-181D were targets of APOC1 and APOE in patients with ACC. These miRNAs are involved in the proliferation, migration, inva- sion, and drug resistance of tumor cells, making them prom- ising targets for cancer treatment.40-42 However, these miRNAs have not been reported to be associated with ACC. ERBB2, TGFBR1, CDK1, MAP2K7, and CDK2 were kinase targets of APOC1 and APOE in patients with ACC. CDK1 serves as a therapeutic target for ACC by regu- lating the epithelial-mesenchymal transition, G2/M phase transition, and PANoptosis.43 CDK2 mRNA expression was strongly upregulated in ACC and CDK inhibitors showed dose-dependent antiproliferative effects. CDK2 mRNA expression is strongly upregulated in ACC and the CDK inhibitor flavopiridol has a dose-dependent anti- proliferative effect.44 ERBB2, TGFBR1, and MAP2K7 might be promising targets for cancer therapy. However, their relationship to ACC remains unexplored. Thus, these transcription factors, miRNAs, and kinases of APOC1, APOE, and the ANGs may be able to treat ACC.

Next, the correlation between APOC1 and APOE expres- sion and the differentially expressed genes in patients with ACC were examined. A correlation was observed between

Figure 4. Genes differentially expressed in correlation with APOC1 and APOE expression in ACC (linkedOmics). (a and d) Pearson test was used to analyze correlations between APOC1, APOE and genes differentially expressed in ACC, respectively; (b, c, e, and f) Heat maps showing genes positively and negatively correlated with APOC1 and APOE in ACC, respectively (TOP 50 genes); The scatter plot shows Pearson correlation of APOC1 and APOE expression with expression of APOE (g), APOC1P1 (h), SPOCK1 (i), APOC1 (j), FAM196B (k), and CAMK1D (l) in ACC; Red indicates positively correlated genes and blue indicates negatively correlated genes.

(a)

APOC1 Association Result

(b)

(c)

APOC1

2

15

-log10(pvalue)

-

10

Z-Score Group 3 f

Z-Score Group 3

0

2

4

a

0

-1

€-3

2

4

-1

2

43

-4

5

0

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

2.0

APOC1

APOC1

Pearson Correlation Coefficient (Pearson test)

(d)

APOE Association Result

(e)

(f)

0

APOE

1

-log10(pvalue)

10

Z-Score Group

-3

Z-Score Group 3

H

E

W 4

N

Jag

2

-1

0

0-3

4

e-3

5

0

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

2.0

APOE

APOE

Pearson Correlation Coefficient (Pearson test)

(g)

Pearson-Correlation:0.7713 P-value:9.06e-17 Sample Size:(N=79)

(h)

Pearson-Correlation:0.6812 P-value:4.872e-12 Sample Size:(N=79)

(i)

Pearson-Correlation :- 0.5938 P-value:8.008e-09 Sample Size:(N=79)

&

6

0

00

APOE

15

APOC1P1

$

SPOCK1

10

2

10

0

5

0

4

6

8

10

12

14

16

4

6

8

10

12

14

16

4

6

8

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12

14

16

APOC1

APOC1

APOC1

(j)

Pearson-Correlation:0.7713 P-value:9.06e-17 Sample Size:(N=79)

(k)

Pearson-Correlation :- 0.5897 P-value:1.076e-08 Sample Size:(N=79)

(I)

Pearson-Correlation :- 0.5669 P-value:5.122e-08 Sample Size:(N=79)

2

10

15

00

0

00

00

APOC1

FAM196B

6

CAMK1D

0

6

10

+

4

2

5

0

0

8

10

12

14

16

18

8

10

12

14

16

18

8

10

12

14

16

18

APOE

APOE

APOE

Figure 5. The correlation between APOC1, APOE expression and immune cell infiltration and anti-PD1/pd-L1 immunotherapy in ACC (BEST). (a and b) Heat maps showing the correlation between APOC1, APOE and immune cell infiltration in ACC, respectively; (c-h) The correlation between APOC1, APOE expression and immunescore in ACC, respectively; (i and j) Boxplot showing the correlation between APOC1, APOE expression and anti-PD1/PD-L1 immunotherapy in ACC, respectively.

(a)

APOC1

(b)

APOE

(c)

GSE10927

1

Monocytic_lineage

Cor - 0.452; Pval - 60-04

Neutrophil

Myeloid_cendrift_clab

Macrophago

Endotheaalco

T_col_Coa

2.

APOC1

FIbr DCLASES

T_cell_CD4

ImmuneScore_estimate

T_cols

Cytotoxic :_ lymphocytes

Cod_T_cells B_lineage

Myeloid_dendritic_cels

Neutrophils

Endothelial cells

Macrophage

CD8_T_cels

Neutrophil DC

B_lineage NK_cells

T_cell_COM

T_cols

T_col_CD8

Cytotoxic_lymphocytes

B_cell

Fibroblasts

Monocytes

CAF$

Macrontome 09 Macrophages_M2

CD4_Ters

APOCI Expression (z-score)

Macrophages Ml

Fangen

Pericytes

1- 20h

CD8_Tcels

(d)

CD4+_naive_T-cells

Macrophages

GSE12368

Sebocytes

Macrophages_M2

Cor = 0.506; Pval = 6.60-03

Mast_cells

DOC

DC

aDc

CD4+_naive_T-cells

APOC1

NIKT

Neutrophils

POC

NKT

CUP

ImmuneScore_estimate

CD4+_Tcm

pro_8-cels

Neutrophils

Mast_cells

Eosinophi

CD4+ T-CUB

Fibroblasts

Class-switched_memory_B-cells

Myocytes CDC

ly_Endothelial cels

Endothelial_cols

Memory_B-cells

naive_B-cells

Sebocytes

Fibroblasts

CMP

MSC

ly_Endothelial_cells

my_Endothelial_cells

mv_Endothelial_cells

MEP

Erythrocytes

GMP

NK_cells

Endothelial_cells

Memory_8-cels

APOCI Expression (z-score)

Hepatocytes

CD4+_memo-

CD4+_memory_T-cells

NES

MSC

Astrocytes

Bascohits

Olenlast

Eosinoohls

(e)

GSE33371

HSC

Adipocytens

Mesancial cels

CD8. Tom

CO4+_Tem

Cor = 0.452: Pval = fe-04

CDC

COB+_Tem

OC

naive_8-cels

2

APOC1

CD8+_T-cells

Chondrocytes

CD8+_T-ceils

CD4+_Tem Platelets

CD8+_naive T-cels

ImmuneScore_estimate

CDB ._ naive_T-cells

Freadi pocylons

Vodafone

CD4. Tom

Keratinocules

CD4+_T-cels

Epithelial cells

Melanocytes

Neurons

Algorithmus

Mendeyters

Chondrocytes

Macrophages

Algorithms

Mesangial cells

Plasma_cells

CIBERSORT ARS CIBERSORT_ABS

Macrophages Mit

FPIC

Smooth_muscle

ESTIMATE

Osteoblast

Thi_cels

ESTIMATE

Neurons

Tgd_cells

Pericytes

pro_B-ccils

Basophils

B-cels

APOC1 Expression (z-score)

1-556 MEP

Correlation

CDB ._ Tom

Preadipocytos

Plasma ces

Correlation

CD8+

3

2

(f)

Skeletal_muscle

Myceyos

Mocyles

0.5

GSE76021

-1

Cor = 0.417; Pval = 2.50-02

Macrophages

Mega Tregs

CD8_Tcells Bcells

Epithelial cells

APOC1

NKcells

CAFS

Adipocytes

Endothelial

Class-switched_memory_8-cells

ImmuneScore_estimate

CD4_Tcells

Smooth_muscle Skeletal_muscle

Monocytes

T_cols_CD8

Macrophages_M2

Dendrioc_cels,resting

Macrophase

NK_cells_activated

Macrophages_MI

T_cells_CD8

POPIS

picanha

B cells naive

Dendritic_cels

Macrophages, M1

T cells samma direita T_cells_folicular_helper

B_cels. THERE

T_cells_CD4

Dendritic_cells_activated

T_cells_regulatory_(Tregs)

NK_cells_resting

APOC1 Expression (z-score)

Neutrophils

B_cells_naive

Mast_cells_resting

Mast_cells_activated

NK_cells_resting

Eosinophils

T_cells_CD4_memory actual

T_cells_CDB

_cells_CD4_naive

T_cells_CD4_memory-activated

T-cells_CD4_nave

(g)

coils memory

T_cells_CD4 members Mo

Macro

GSE90713

Cor - 0.318; Pval = 1.50-02

Mast_cells_activated

Mast cells resting

Macrophages Mi

Dendres see

Monocytes

Macrophages M2

T_cells follicular_helper

APOE

Dendritic_cells_resting

T_cells_regulatory_(Tregs)

T_cells_gamma_delta

ImmuneScore_estimate

Eosinophils

T_cells_CO8

Dendritic_cells_activated

Monocytes

NK_cells_activated

T_cells_CD4_memory_resting

Plasma_cells

B_cells_naive

Macrophages_MO

Plasma cells

T_cells gamma carta

Ces Folicular_helper

B_cols_memory

Tensemanales med

T_cells_regulatory-Mapy

AR CHIS Testing

Mas este

ocels_nave

T_cells_CD4 naive

@ cells memory

Macrophages Mi NK_cells resting

Masz_cols_resting

Mast_cells_activated

T_cells_CD4_memory_activated

Macrophages_M1

Eosinophils

APOE Expression (z-score)

T_cells_CD4_memory_resting

T_calls_CD8

Mast_cells_activated

Macrophages_MO

T_cells_CD4_memory_activated

T_cells_CD4_naive

(h)

Macrophages_M1

Monocytes

T_cells_CD4_memory_resting

GSE143383

Comment_COB

Cor = 0.307; Pval = 20-02

T cells Che T_cells_CD8

cophages 0

B cells mummery

Dendro mie

NOCH

T cells folictre beleer

APOE

T_cels_regulatory_(Tregs)

T ents aumma dela

ImmuneScore_estimate

Plasma_cols

T_cells_CD4

Dendritic_cells_activoned

ImmuneScore

ESTIMATE Score

GSE90713

GSE 143383

StromalScore ESTIMATEScore ImmuneScore

StromalScore

GSE33371

CSE10927

GSE19775

GSE90713

TCLA

GSE143383

(i)

(j)

APOE Expression (z-score)

Amato cohort 2020

Kim cohort 2019

Anti-PD-1

Anti-PD-1/PD-L1

T-test, p = 0.01

APOC1

2

T-test, p = 0.05

APOE

1

APOC1 Expression (z-score)

APOE Expression (z-score)

0

0

-1

$1

NR

R

NR

R

Response

Response

Figure 6. IC50 evaluation of pilaralisib and elesclomol in different tissue types of cancer (BEST and genomics of drug sensitivity in cancer). (a and f) Heat maps showing APOC1 and APOE high expression indicates resistance drugs ranking, respectively; (b) Cell line IC50 values of pilaralisib; (c) Cell line AUC values of pilaralisib; (d) SW13 cell line IC50 values of pilaralisib; (e) SW13 cell line AUC values of pilaralisib. (g) Cell line IC50 values of elesclomol; (h) Cell line AUC values of elesclomol; (i) SW13 Cell line IC50 values of elesclomol; (j) SW13 cell line AUC values of elesclomol.

(a)

(b)

(c)

Cell line IC50 values (Pilaralisib)

Cell line AUC values (Pilaralisib)

10ª

Number of cell lines screened : 919

1.0

Number of cell lines screened : 919

Pilaralisib_372

Maximum IC50 (uM) : 740

Maximum AUC : 0.988

High expression indicates resistance

Kobe2602_563

Geometric mean (uM) : 26.3

Minimum AUC : 0.374

Minimum IC50 (uM) : 0.573

SB52334_304

102

Min screening concentration (uM) : 0.0781

0.8

Motesanib_1029

Max screening concentration (uM) : 20 0

C-75_435

GSK1904529A_202

IC50 (micromolar)

-max conc

LFM-A13_192

10’

CD532_449

0.6

Capivasertib_1136

AUC

ARRY-520_474

Elesclomol_1031

10°

TW 37_1149

Correlation

0.4

JNJ38877605_284

SB505124_476

0.5

CI-1033_362

0

BMS-754807_184

-0.5

10-1

-1

-min cone

0.2

GSE12368

GSE143383

GSE90713

GSE76019

GSE33371

GSE10927

GSE76021

GSE19750

TCGA_ACC

102

ranked by sensitivity

0.0

ranked by sensitivity

(d)

(e)

Cell line (SW13) IC50 values (Pilaralisib)

Cell line (SW13) AUC values (Pilaralisib)

103

1.0

IC50 (µM) : 16.488967

AUC : 0.857985

102

0.8

IC50 (micromolar)

max conc

10

0.6

AUC

10°

0.4

10

-min cone

0.2

102

0.0

ranked by sensitivity

ranked by sensitivity

(f)

(g)

(h)

Cell line IC50 values (Elesclomol)

Cell line AUC values (Elesclomol)

102

1.0

Number of cell lines screened : 921

Number of cell lines screened : 921

High expression indicates resistance

Elesclomol_1031

Maximum IC50 (uM) : 10.3

Geometric mean (uM) : 0.0486

Maximum AUC : 0.997

IGFR_3801_1430

10

Minimum IC50 (uM) : 0.000231

Minimum AUC : 0.0209

ARRY-520_474

Min screening concentration (uM) : 0.000781

0.8

FGFR_3831_1422

Max screening concentration (uM) : 0.200

Talazoparib_1259

IC50 (micromolar)

10°

PARP_9482_1460

0.6

PARP_0108_1459

- max cone

10

AUC

PI-103_302

PLK_6522_1451

AZD4877_1409

Correlation

0.4

10-2

AZD8186_1444

0.5

AZD6482_156

0

EphB4_9721_1413

-0.5

10-

-min conc

0.2

-1

GSE143383

GSE90713 TCGA_ACC

GSE19775

10-

ranked by sensitivity

0.0

ranked by sensitivity

(i)

(j)

Cell line (SW13) IC50 values (Elesclomol)

Cell line (SW13) AUC values (Elesclomol)

102

1.0

IC50 (u.M) :0.007628

AUC : 0.421688

10”

0.8

IC50 (micromolar)

10°

0.6

max conc

10-

AUC

102

0.4

104

min cone

0.2

10-

ranked by sensitivity

0.0

ranked by sensitivity

APOC1 and APOE expression and 4471 and 4021 genes, respectively. Among them, APOC1P1 was positively and SPOCK1, FAM196B, CAMK1D were negatively correlated with the expression of APOC1 and APOE. Thus, targeting these genes may provide additional therapies for ACC. Immune infiltration of tumors plays a key role in tumor pro- gression and outcome.45 Cancer immunotherapy has led to significant advances in the treatment of multiple cancers. As expected, immune cell infiltration was positively correlated with APOC1 and APOE expression levels in patients with ACC. Expression levels of APOC1 and APOE in tumors are closely related to the infiltration of various immune cells. In addition, APOC1 and APOE were significantly co-expressed with immunomodulatory genes. APOC1 and APOE are immunological biomarkers that are associated with the infiltration of various immune cells.13,46 Furthermore, APOC1 and APOE were strongly downregu- lated and upregulated, respectively, in patients with ACC that were treated with anti-PD1/PD-L1. In patients with ACC, targeting APOC1, APOE, or the related regulatory targets may improve the immune microenvironment. Patients with ACC that are treated with anti-PD1 may have a better prognosis. Furthermore, the inhibitory effects of pilaralisib and elesclomol on ACC lines were investigated. Pilaralisib and elesclomol inhibited the expression in 919 and 921 cancer cell lines, respectively. Among them, elesclomol had a stronger inhibitory effect on the SW13 cell line than that of pilaralisib. In summary, pilaralisib and elesclomol inhibited cancer cells on a broad spectrum. The phosphoinositide 3-kinase inhibi- tor pilaralisib and the HSP90 inhibitor elesclomol have good antitumor effects and are safe. However, the effect of pilaralisib and elesclomol on ACC remains unclear. Thus, pilaralisib, elesclomol, and inhibitors of APOC1 and APOE or their regulatory targets, may be important strategies for the treatment of ACC. We identified the roles of APOC1 and APOE in ACC using bioinformatics methods. However, further validation through in vitro and ex vivo experiments is necessary to confirm their relationship.

In conclusion, this study preliminarily clarified that APOC1 and APOE might be potential therapeutic and prog- nostic targets for ACC and provided insights into the mech- anism and treatment of ACC.

ORCID iD

Yongli Situ ID https://orcid.org/0000-0003-2244-115X

Statements and declarations

Author contributions

INTERPRETATION OR ANALYSIS OF DATA: SL and YS.

PREPARATION OF THE MANUSCRIPT: SL and SX.

REVISION FOR IMPORTANT INTELLECTUAL CONTENT:

SL, SX and YS. SUPERVISION: YS.

Funding

This research was funded by postdoctoral Foundation of Guangdong Medical University (4SG22292G).

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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