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CDH2 and CDH13 as potential prognostic and therapeutic targets for adrenocortical carcinoma

Yongli Situ Da*, Li Denga*, Ziqing Huangª, Xiaoli Jianga,b, Liubing Zhaoa,b, Juying Zhanga,b, Lingling Lua, Quanyan Lianga,b, Qinying Xuª, Zheng Shaoª, and Meng Lianga

ªDepartment of Parasitology, Guangdong Medical University, Zhanjiang, China; bSchool of Medical Technology, Guangdong Medical University, Dongguan, China

ABSTRACT

Cadherin 2 (CDH2, N-cadherin) and cadherin 13 (CDH13, T-cadherin, H-cadherin) affect the progress and prognoses of many cancers. However, their roles in adrenocortical carcinoma (ACC), a rare endocrine cancer, remain unclear. To decipher the roles of these proteins in ACC and to identify their regulatory targets, we analyzed their expression levels, gene regulatory networks, prognostic value, and targets in ACC, using various bioinformatic analyses. CDH2 was strongly downregulated and CDH13 was strongly upregulated in patients with ACC; the expression levels of these genes affected the prognosis. In 75 patients, the expression of CDH2 and CDH13 was altered by 8% and 5%, respectively. CDH2 and CDH13, as well as their neighboring genes, were predicted to form a complex network of interactions, mainly through coexpression and physical and genetic interactions. CDH2 and its altered neighboring genes (ANGs) mainly affect tumor-related gene expression, cell cycle, and energy metabolism. The regulation of tumor-related integrin function, gene transcription, metabolism, and amide and phospholipid metabo- lism are the main functions of CDH13 and its ANGs. MiRNA and kinase targets of CDH2 and CDH13 in ACC were identified. CDH13 expression in patients with ACC was positively associated with immune cell infiltration. Anti-PD1/CTLA-4/PD-L1 immunotherapy significantly downregulated the expression of CDH13 in patients with ACC. Foretinib and elesclomol were predicted to exert strong inhibitory effects on SW13 cells by inhibiting the expression of CDH2 and CDH13. These data indicate that CDH2 and CDH13 are promising targets for precise treatment of ACC and may serve as new biomarkers for ACC prognosis.

ARTICLE HISTORY

Received 21 May 2023 Revised 29 July 2024 Accepted 7 November 2024

KEYWORDS

Bioinformatics; cadherin; cancer; cell cycle; gene regulatory network; immunotherapy; prognosis; transcription

Introduction

Adrenocortical carcinoma (ACC) is a rare endocrine tumor with a global incidence of 0.7-2.0 cases/million/year.1 Approximately 60% of ACC cases are functional. There is a wide range of clinical syndromes depending on the type of hormones produced.2 The prognosis of patients with ACC is poor, with a 5-year survival rate of < 40%.3 For most patients, there is no effective treatment to prolong survival, and complete surgical resection is the only treatment option.4 Therefore, it is necessary to determine the mechanisms underlying the occurrence and development of ACC, and to identify therapeutic targets.

Cadherin is a tumor suppressor that regulates tissue devel- opment and differentiation. Currently, more than 100 cadherins are identified, which are categorized into four groups, namely classical cadherins, protocadherins, desmosomal cadherins, and cadherin-related proteins.5 Increasing evidence suggests that an imbalance in cadherin expression caused by gene alterations can lead to tumor growth, invasion, and metastasis.6,7 Cadherin 2 (CDH2, N-cadherin) is a member of the classical cadherin group that maintains the integrity of cells and participates in many signal transduction pathways. Abnormal expression of CDH2 has been reported in many cancers, including that of

the lung, breast, and prostate, as well as squamous cell carcinoma.7 Abnormal expression of CDH2 can regulate the progression of malignant tumors by affecting apoptosis, angio- genesis, invasion, and metastasis of tumor cells.8 Therefore, CDH2 may be used as a therapeutic target and prognostic biomarker for multiple tumors.9 Cadherin 13 (CDH13, T-cadherin, H-cadherin) is a new member of the cadherin superfamily that maintains normal tissue structure. Abnormalities in CDH13 have been observed in many types of human malignant tumors.10 Recently, CDH13 has been shown to play a role as an anticancer gene in lung, breast, ovary, bladder, and gastric cancer.11,12 Abnormal expression of CDH13 plays a key role in cancer development by promoting the inactivation of tumor suppressor genes, activation of onco- genes, and increasing chromosome instability.13

The roles of CDH2 and CDH13 in ACC are not well under- stood. Therefore, in this study, we systematically analyzed the expression, gene regulatory network, prognostic value, puta- tive targets, and potential therapeutic agents of CDH2 and CDH13 in patients with ACC. Moreover, we examined the association of ACC with CDH2 and CDH13 and identified potential new targets and drugs for ACC therapy.

CONTACT Zheng Shao shaozheng@gdmu.edu.cn; Meng Liang East Wenming Road, Xiashan District, Zhanjiang 524023, China

15848164641@163.com

Department of Parasitology, Guangdong Medical University, No. 2

*These authors contributed equally: Yongli Situ and Li Deng.

@ 2024 The Author(s). Published with license by Taylor & Francis Group, LLC.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The terms on which this article has been published allow the posting of the Accepted Manuscript in a repository by the author(s) or with their consent.

Results

CDH2 and CDH13 expression, prognosis, and genetic alterations in ACC

The transcript level of CDH2 was significantly downregulated (p < .05; Figure 1a) and that of CDH13 was significantly upre- gulated (p <. 05; Figure la-f) in patients with ACC. CDH2 transcript levels were significantly lower in males than in

females (p <. 05; Figure 1g,h). The transcript levels of both genes were significantly downregulated in older patients (≥65 years) compared with those in younger patients (<65 years) (p < .05; Figure 1i,j). Furthermore, the overall survival was longer for patients with ACC exhibiting low CDH2 expres- sion than for those with high expression (p =. 041; Figure 1k). Disease-free survival was longer in patients with ACC having low CDH2 and CDH13 expression than in those with high

Figure 1. The transcription levels, prognostic value, and genetic alteration of CDH2 and CDH13 in adrenocortical carcinoma (ACC). (a) Boxplot showing transcription level of CDH2 in patients with ACC (GEPIA); (b) Boxplot showing transcription level of CDH13 in patients with ACC (GEPIA); (c - f) Boxplot showing transcription level of CDH13 in patients with ACC (BEST); (g and h) Boxplot showing transcription level of CDH2 in patients with ACC based on sex (UALCAN and BEST); (i) Boxplot showing transcription level of CDH2 in patients with ACC based on age (BEST); (j) boxplot showing transcription level of CDH13 in patients with ACC based on age (BEST); (k) the overall survival curve of CDH2 in patients with ACC (GEPIA); (I) the disease-free survival curve of CDH2 in patients with ACC (GEPIA); (m) the overall survival curve of CDH13 in patients with ACC (GEPIA); (n) the disease-free survival curve of CDH13 in patients with ACC (GEPIA); (o) Genetic alteration of CDH2 in patients with ACC (cBioportal); (p) Genetic alteration of CDH13 in patients with ACC (cBioportal); * p <. 05.

a

b

C

d

e

GSE33371

f

CDH2

CDH13

GSE10927

GSE143383

GSE90713

0

Wilcoxon, p = 0.00061

T-test, p = 0.00046

Wilcoxon, p = 0.00061

T-test, p = 5.20-05

00

4

CDH13

CDH13

·

4

CDH13

2-

2

CDH13

4

CDH13 Expression (z-score)

CDH13 Expression (z-score)

CDH13 Expression (z-score)

CDH13 Expression (z-score)

6

1

1

3

2

2

0

-

0

N

1

0

1

0

2

-

-2

-2

-3

0

0

Normal

Tumor

Normal

Tumor

Normal

Tumor

Normal

Tumor

(num(T)-77; num(N)-12B)

ACC

(num(T)-77; num(N)-12B)

ACC

Tissue

Tissue

Tissue

Tissue

09

h

TCGA_ACC

1

TCGA_ACC

j

GSE19775

Expression of CDH2 in ACC based on patient’s gender

Wilcoxon, p = 0.0076

Wilcoxon, p = 0.0014

2

Wilcoxon, p = 0.014

80

2

CDH2

2

CDH2

CDH13

DE

CDH2 Expression (z-score)

CDH2 Expression (z-score)

CDH13 Expression (z-score)

1

.

Transcript per million

60

1

1

0

40

.

0

-1

0

20

-2

0

-1

Male

(n=31)

Female (n=48)

-1

TCGA samples

Female

Male

65

-3

>65

$65

,65

Gender

Age

Age

k

1

Overall Survival

Disease Free Survival

m

Overall Survival

n

Disease Free Survival

0

Low CDH2 TPM

0

High CDH2 TPM

Low CDH2 TPM

10

9

High TPM

Low CDH13 TPM

High CDH13 TPM

Low CDH13 TPM

Logrank p=0.0061

Logrank p=0.064

High CDH13 TPM

Logrank p=0.041

HR(high)=2.2

HR(high)=2.6

HR(high)=2.1

Logrank p=0.00027

0.8

p(HR)-0.047

0.8

p(HR)-0.0083

0.8

0g

HR(high)=3.5

p(HR)-0.00059

Percent survival

n(high)=38

Percent survival

n(high)=38

p(HR)-0.07

n (low)=38

n[low)=38

Percent survival

n(high)=38 n(low)=38

Percent survival

n(high)=38 nilow) 30

0.6

0.6

0.6

0.6

0.4

0.4

0.4

0.4

2

2

2

8

CDH2

CDH2

CDH13

8

8

8

CDH13

8

0

50

100

150

0

50

100

150

0

50

100

150

0

50

100

150

Months

Months

Months

Months

0

Altered in 6 (8%) of 75 patients

CHD2

8%

Genetic alteration

Missense Mutation (unknown significance)

Amplification

mRNA High

mRNA Low

No alterations

p

Altered in 4 (5%) of 75 patients

CDH13

5%

Genetic alteration

Deep Deletion

mRNA Low

No alterations

expression of these genes (p =. 0061 and p = . 00027, respec- tively; Figures 11-n). Moreover, CDH2 and CDH13 expression was altered by 8% and 5%, respectively, in patients with ACC (Figure 1o,p).

Interaction network of CDH2 and CDH13 and their altered neighboring genes in ACC

We noted CDH2 and CDH13 altered neighboring gene (ANG) alteration frequencies of ≥ 33.33% and ≥ 25.00%, respectively, among the 50 most frequent ANGs in patients with ACC (Tables 1 and 2). The most frequent ANGs for CDH2 in patients with ACC were PKHD1 (66.67%), PHF20L1 (50.00%), and KCNH7 (50.00%) (Table 1). Furthermore, NT5C3A (50.00%), ANKMY1 (50.00%), and CD1C (50.00%) were the most frequent ANGs of CDH13 in patients with ACC (Table 2). We obtained 43 nodes and 124 edges in the protein - protein interaction (PPI) networks of CDH2 and ANGs (Figure 2a). CDH2 was predicted to be connected to ANGs by coexpression, shared protein domains, colocalization, phy- sical interactions, and genetic interactions in a complex inter- action network (Figure 2b). Moreover, we obtained 40 nodes and 114 edges in the PPI networks of CDH13 and ANGs (Figure 2c). CDH13 was predicted to be connected to ANGs by coexpression, physical interactions, pathways, colocaliza- tion, shared protein domains, and genetic interactions (Figure 2d).

Gene ontology function and Kyoto encyclopedia of genes and genomes pathway enrichment analysis of CDH2, CDH13, and their ANGs in ACC

The biological processes associated with CDH2 and ANGs in patients with ACC were mainly associated with cell morphogenesis, epithelial cell development, postsynaptic organization, microtubule cytoskeleton organization, sper- matid development, cellular calcium ion homeostasis, brain development, camera-type eye development, and purine ribonucleotide metabolism (Figure 2e). Moreover, glutama- tergic synapses, presynaptic active zones, endoplasmic reti- culum lumen, dendrites, and the mitochondrial matrix were the main cellular components of CDH2 and its ANGs in patients with ACC (Figure 2f). The molecular functions of CDH2 and its ANGs in patients with ACC included struc- tural constituents of synapses, histone deacetylase binding, monoatomic ion transmembrane transporter activity, ATP hydrolysis activity, protein kinase binding, calcium ion binding, and protein homodimerization activity (Figure 2g). The biological processes related to CDH13 and its ANGs in patients with ACC were T-cell activation involved in immune, monocarboxylic acid metabolic pro- cess, regulation of fat cell differentiation, phagocytosis, epithelial cell proliferation, positive regulation of binding, regulation of lipid biosynthetic process, small GTPase- mediated signal transduction, muscle organ development, secretion by cell, plasma membrane-bound cell projection assembly, DNA metabolic process, synaptic signaling, and positive regulation of cell adhesion (Figure 2h). Additionally, the main cellular components of CDH13 and

Table 1. The top 50 of CDH2 neighbor gene alterations in ACC (cBioportal).
GeneAltered groupUnaltered groupp-Value
DNMT3L3 (50.00%)0 (0.00%)2.96E-04
PKHD14 (66.67%)5 (7.25%)1.38E-03
PHF20L13 (50.00%)0 (0.00%)2.96E-04
KCNH73 (50.00%)1 (1.45%)1.15E-03
CHD53 (50.00%)2 (2.90%)2.78E-03
DROSHA3 (50.00%)2 (2.90%)2.78E-03
ZSWIM63 (50.00%)2 (2.90%)2.78E-03
ADAMTSL43 (50.00%)3 (4.35%)5.38E-03
ATP2B33 (50.00%)3 (4.35%)5.38E-03
MAGEA123 (50.00%)3 (4.35%)5.38E-03
MAGEA23 (50.00%)3 (4.35%)5.38E-03
MAGEA2B3 (50.00%)3 (4.35%)5.38E-03
OPLAH3 (50.00%)3 (4.35%)5.38E-03
TRIO3 (50.00%)3 (4.35%)5.38E-03
ACTN12 (33.33%)0 (0.00%)5.41E-03
AMTN2 (33.33%)0 (0.00%)5.41E-03
ANKRD18DP2 (33.33%)0 (0.00%)5.41E-03
ANXA132 (33.33%)0 (0.00%)5.41E-03
ARTN2 (33.33%)0 (0.00%)5.41E-03
ATP13A52 (33.33%)0 (0.00%)5.41E-03
ATP5PB2 (33.33%)0 (0.00%)5.41E-03
BDH12 (33.33%)0 (0.00%)5.41E-03
BSN2 (33.33%)0 (0.00%)5.41E-03
C8B2 (33.33%)0 (0.00%)5.41E-03
C8ORF762 (33.33%)0 (0.00%)5.41E-03
CCDC392 (33.33%)0 (0.00%)5.41E-03
CHPT12 (33.33%)0 (0.00%)5.41E-03
CHRD2 (33.33%)0 (0.00%)5.41E-03
CLCN22 (33.33%)0 (0.00%)5.41E-03
COPB22 (33.33%)0 (0.00%)5.41E-03
DLG12 (33.33%)0 (0.00%)5.41E-03
DRGX2 (33.33%)0 (0.00%)5.41E-03
ECHS12 (33.33%)0 (0.00%)5.41E-03
ELAVL22 (33.33%)0 (0.00%)5.41E-03
ENAM2 (33.33%)0 (0.00%)5.41E-03
FAM157A2 (33.33%)0 (0.00%)5.41E-03
FAM83A2 (33.33%)0 (0.00%)5.41E-03
FAM91A12 (33.33%)0 (0.00%)5.41E-03
FAT32 (33.33%)0 (0.00%)5.41E-03
FBXO322 (33.33%)0 (0.00%)5.41E-03
FGGY2 (33.33%)0 (0.00%)5.41E-03
FRG2B2 (33.33%)0 (0.00%)5.41E-03
FYTTD12 (33.33%)0 (0.00%)5.41E-03
HJURP2 (33.33%)0 (0.00%)5.41E-03
HOOK12 (33.33%)0 (0.00%)5.41E-03
HSD17B42 (33.33%)0 (0.00%)5.41E-03
IFI44L2 (33.33%)0 (0.00%)5.41E-03
IGF2BP22 (33.33%)0 (0.00%)5.41E-03
IL18R12 (33.33%)0 (0.00%)5.41E-03
IQCG2 (33.33%)0 (0.00%)5.41E-03

its top 50 ANGs in patients with ACC were the transcrip- tion repressor complex, synaptic membrane, actin-based cell projection, extracellular matrix, plasma membrane protein complex, and transporter complex (Figure 2i). The molecu- lar functions of CDH13 and its ANGs in patients with ACC included integrin binding, transcription corepressor activity, ATP-dependent activity, amide binding, and phospholipid binding (Figure 2j).

MiRNA and kinase targets of CDH2 and CDH13 in patients with ACC

Using LinkedOmics, we found the miRNA targets of CDH2 and CDH13 (Table 3). MiR-331, miR-486, and miR-24 were the targets of CDH2 in ACC (p <. 001) (Table 3). The miRNA targets of CDH13 in ACC were miR-101, miR-142-3P, and miR-527 (p <. 001) (Table 3). Moreover, we found that

Table 2. The top 50 of CDH13 neighbor gene alterations in ACC (cBioportal).
GeneAltered groupUnaltered groupp-Value
NT5C3A2 (50.00%)0 (0.00%)2.16E-03
ANKMY12 (50.00%)1 (1.41%)6.37E-03
CD1C2 (50.00%)1 (1.41%)6.37E-03
CHD22 (50.00%)1 (1.41%)6.37E-03
CTBP22 (50.00%)1 (1.41%)6.37E-03
FCRL22 (50.00%)1 (1.41%)6.37E-03
TRIB32 (50.00%)1 (1.41%)6.37E-03
ECHDC32 (50.00%)2 (2.82%)0.0125
NDUFA102 (50.00%)2 (2.82%)0.0125
PTPDC12 (50.00%)2 (2.82%)0.0125
SCARB12 (50.00%)2 (2.82%)0.0125
CCN22 (50.00%)4 (5.63%)0.0301
ENTREP32 (50.00%)4 (5.63%)0.0301
FAM241A2 (50.00%)4 (5.63%)0.0301
MEX3C2 (50.00%)4 (5.63%)0.0301
NOM12 (50.00%)4 (5.63%)0.0301
TUT72 (50.00%)4 (5.63%)0.0301
ZFPM12 (50.00%)4 (5.63%)0.0301
GRIN3B2 (50.00%)5 (7.04%)0.0413
MYO1G2 (50.00%)5 (7.04%)0.0413
UTRN2 (50.00%)5 (7.04%)0.0413
ACBD51 (25.00%)0 (0.00%)0.05
ADAM231 (25.00%)0 (0.00%)0.05
AOAH1 (25.00%)0 (0.00%)0.05
APBB1IP1 (25.00%)0 (0.00%)0.05
ARL5B1 (25.00%)0 (0.00%)0.05
ASB11 (25.00%)0 (0.00%)0.05
ATP8B11 (25.00%)0 (0.00%)0.05
BAMBI1 (25.00%)0 (0.00%)0.05
BMPER1 (25.00%)0 (0.00%)0.05
BMT21 (25.00%)0 (0.00%)0.05
BTD1 (25.00%)0 (0.00%)0.05
C11ORF681 (25.00%)0 (0.00%)0.05
C7ORF331 (25.00%)0 (0.00%)0.05
CCN61 (25.00%)0 (0.00%)0.05
CCR41 (25.00%)0 (0.00%)0.05
CDC401 (25.00%)0 (0.00%)0.05
CEP701 (25.00%)0 (0.00%)0.05
CHD41 (25.00%)0 (0.00%)0.05
CHML1 (25.00%)0 (0.00%)0.05
CMC21 (25.00%)0 (0.00%)0.05
CNBP1 (25.00%)0 (0.00%)0.05
DCUN1D41 (25.00%)0 (0.00%)0.05
DGKI1 (25.00%)0 (0.00%)0.05
DHCR71 (25.00%)0 (0.00%)0.05
DPY19L11 (25.00%)0 (0.00%)0.05
DPY19L2P11 (25.00%)0 (0.00%)0.05
DRAP11 (25.00%)0 (0.00%)0.05
EEPD11 (25.00%)0 (0.00%)0.05
ELMO11 (25.00%)0 (0.00%)0.05

DYRK1B, LYN, and NLK were the kinase targets of CDH2 in patients with ACC (p <. 001) (Table 4). The kinase targets of CDH13 were TTK, CDK2, and CHEK1 in patients with ACC (p <. 001) (Table 4).

Correlation of differentially expressed genes and CDH2 and CDH13 expression in patients with ACC

A total of 4,824 and 2,748 genes were found to be closely related to CDH2 and CDH13, respectively, in patients with ACC (Figure 3a-d). Among them, 2,096 and 1,898 genes showed positive correlation and 2,728 and 850 genes showed negative correlation with CDH2 and CDH13 expression, respectively (Figure 3a-d). Fifty genes showed significant positive and nega- tive correlation with CDH2 and CDH13 expression in patients with ACC (Figure 3b,c,e,f). The expression of CDH2 was strongly and positively associated with VSNL1 (Pearson correlation

coefficient [PCC] = 0.6735, p= 1.043e-11; Figure 3g), TCF7 (PCC=0.6491, p = 9.77e-11; Figure 3h), and RASL10B (PCC= 0.6475, p = 1.128e-10; Figure 3i). The expression of CDH13 was positively correlated with COL4A1 (PCC = 0.7066, p = 3.436e-13; Figure 3j), ANGPT2 (PCC= 0.693, p = 1.478e-12; Figure 3k), and ESAM (PCC = 0.6546, p = 5.997e-11; Figure 3l) expression.

Correlation of immune cell infiltration and CDH13 expression and anti-PD1/CTLA-4/PD-L1 immunotherapy in ACC

The expression levels of CDH13 in patients with ACC were positively associated with immune cell infiltration (B cells, CD4+ T cells, macrophages, neutrophils, and dendritic cells) (p <. 05; Figure 4a-f). The cumulative survival of patients with ACC was longer than that of patients with low CD8+ T-cell expression levels (p = . 05; Figure 4b). However, the cumulative survival of patients with ACC was longer in those with low CDH13 expression levels (p =. 04; Figure 4b). Moreover, CDH13 expression in patients with ACC treated with anti- PD1/CTLA-4, anti-PD1PD-L1, and anti-PD-L1 was signifi- cantly downregulated (p = . 05) (Figure 4g-i).

Therapeutic drugs of CDH2 and CDH13 in ACC

Using the BEST database, we predicted foretinib and elesclo- mol as the top drug candidates for targeting CDH2 and CDH13, respectively (Figure 5a-f). Next, the genomics of drug sensitivity in the cancer database was used to evaluate the inhibitory effects of foretinib and elesclomol on an ACC cell line (SW13). Foretinib inhibited 953 cell lines with area under the curve (AUC) values greater than 0.980 (Figure 5c) and had a good inhibitory effect on these cell lines (0.00285 ≤ IC50 [uM] ≤ 3120) (Figure 5b). Furthermore, foretinib had a strong inhibitory effect on SW13 (an ACC cell line) (AUC = 0.783, IC50 [uM] = 3.25) (Figure 5d,e). However, elesclomol inhibited 921 cell lines, with AUC values greater than 0.0209 (Figure 5h) and had a good inhibitory effect on these cell lines (0.000231 ≤ IC50 [uM] ≤ 10.3) (Figure 5g). Elesclomol had a strong inhibitory effect on SW13 cells (AUC= 0.422, IC50 [uM] = 0.00763) (Figure 5i,j).

Discussion

Abnormal expression of CDH13 has been reported in various tumors. However, its expression in patients with ACC remains unknown. CDH13 expression is often downregulated in cancer cells. Low CDH13 expression is associated with poor prognosis in various cancers, such as lung, ovarian, cervical, and prostate cancer.13 Notably, we found that the expression of CDH13 was strongly upregulated in patients with ACC, and low expression was related to a good prognosis in ACC patients. However, CDH2 expression was strongly downregulated in patients with ACC. Downregulation of N-cadherin has been reported in ACC.14 CDH2 expression levels were lower in male patients than in female patients with ACC; patients exhibiting low CDH2 expression had longer survival times than those with high expression. The number of female patients with ACC generally exceeds that of male patients (1.5:1).15,16 Whether

Figure 2. Interaction and function analyses of CDH2, CDH13, and their altered neighboring genes (ANGs) in adrenocortical carcinoma (ACC). (a) Protein - protein interaction (PPI) network of CDH2 and its ANGs in patients with ACC (STRING); (b) Network analyses of CDH2 and its ANGs in patients with ACC (GeneMANIA); (c) PPI network of CDH13 and its ANGs in patients with ACC (STRING); (d) network analyses of CDH13 and its ANGs in patients with ACC (GeneMANIA); (e) biological processes of CDH2 and its ANGs in patients with ACC (Metascape); (f) cellular components of CDH2 and its ANGs in patients with ACC (Metascape); (g) molecular functions of CDH2 and its ANGs in patients with ACC (Metascape); (h) biological processes of CDH13 and its ANGs in patients with ACC (Metascape); (i) cellular components of CDH13 and its ANGs in patients with ACC (Metascape); (j) Molecular functions of CDH13 and its ANGs in patients with ACC (Metascape).

a

C8B

CHRD

b

2

HOOK1

E

HSD17B4

ATP1345

ADAMTSL4

ACTN1

CLCN2

O

BOH1

FGGY

%

ANXA13

TRIO

ATP2B3

COPB2

8

MAGEA2

ECHS1

4p

DLG1

CHPT1

MAGEA12

MAGEAZB

3

*

5

CDH2

ATP5F1

DROSHA

ARTN

CHO5

ENAI

T

DNMT3L

AMTN

FAT3

1

4

“X

ELAVLZ

PHF20L1

IL18R1

IGF2BP2

-

2

BSN

A

Networks

FAM83A

FAM91A1

IFI44L

Co-expression

ZSWIM6

IQCG

CCDC39

Shared protein domainis

OPLAH

Co-localization

C8orf76

FBXO32

2

Physical Interactions

a

$

CDH2

Genetic Interactions

CDH2

DRAP1

CTBP2

CHD2

C

1

2

C110068

CDH13

d

ADAM23

DPY19L1

CHD4

EEPD1

E

ZFPMI

NOM1

DGKI

AMy

5

CDC40

7

BMPER

ELMO1

TRIB3

0

U

C7orf60

UTRN

DHCR7

8

BAMBI

=

SCARB1

CNBP

ACBD5

ČTGF

DCUN1D4

a

AOAH

PTPDC1

PP

ARL58

ZCCHC6

Networks

CHML

Predicted

MYO1G

GRIN3B

Co-expression

ATP9B1

el

Physical Interactions

IA

MEX3C

APBB1IP

CCR4

Pathway

4

BTD

Co-localization

Shared protein domains

FCRL2

CD1C

CEP70

A

CDH13

Genetic Interactions

CDH13

e

CDH2

Biological processes

f

CDH2

Cellular components

GO:0000902: cell morphogenesis

GO:0002064: epithelial cell development

GO:0099173: postsynapse organization

GO:0098978: glutamatergic synapse

GO:0000226: microtubule cytoskeleton organization

GO:0048786: presynaptic active zone

GO:0007286: spermatid development

GO:0005788: endoplasmic reticulum lumen

GO:0006874: cellular calcium ion homeostasis

GO:0007420: brain development

GO:0030425: dendrite

GO:0043010: camera-type eye development

GO:0005759: mitochondrial matrix

GO:0009150: purine ribonucleotide metabolic process

0.0

0.5

1.0

1.5

2.0

2.5

3.0

3.5

4.0

0.0

0.5

1.0

1.5

2.0

2.5

3.0

3.5

4.0

-log10(P)

-log10(P)

h

g

CDH13

CDH2

Biological processes

Molecular functions

GO:0002286: T cell activation involved in immune response

GO:0032787: monocarboxylic acid metabolic process

GO:0098918: structural constituent of synapse

GO:0045598: regulation of fat cell differentiation GO:0006909: phagocytosis

GO:0042826: histone deacetylase binding

GO:0050673: epithelial cell proliferation

GO:0015075: monoatomic ion transmembrane transporter activity

GO:0051099: positive regulation of binding

GO:0016887: ATP hydrolysis activity

GO:0046890: regulation of lipid biosynthetic process

GO:0019901: protein kinase binding

GO:0007264: small GTPase mediated signal transduction

GO:0005509: calcium ion binding

GO:0007517: muscle organ development

GO:0042803: protein homodimerization activity

GO:0032940: secretion by cell

GO:0120031: plasma membrane bounded cell projection assembly

0

1

2

3

4

5

GO:0006259: DNA metabolic process

-log10[P)

GO:0099536: synaptic signaling

GO:0045785: positive regulation of cell adhesion

0.0

0.5

1.0

1.5

2.0

2.5

3.0

3.5

-log10(P)

1 CDH13

Cellular components

j

CDH13

Molecular functions

GO:0017053: transcription repressor complex

GO:0097060: synaptic membrane

GO:0005178: integrin binding

GO:0098858: actin-based cell projection

GO:0003714: transcription corepressor activity

GO:0031012: extracellular matrix

GO:0140657: ATP-dependent activity

GO:0098797: plasma membrane protein complex

GO:0033218: amide binding

GO:1990351: transporter complex

GO:0005543: phospholipid binding

0.0

0.5

1.0

1.5

2.0

2.5

3.0

3.5

0.0

0.5

1.0

1.5

2.0

2.5

3.0

3.5

4.0

-log10(P)

-log10(P)

Table 3. The top three miRNA target of CDH2 and CDH13 in ACC (LinkedOmics).
GeneGene setLeading Edge NumberP-value
CDH2CCAGGGG,miR-33127<2.2e-16
GTACAGG,miR-48613<2.2e-16
CTGAGCC,miR-2445<2.2e-16
CDH13GTACTGT,miR-10165<2.2e-16
ACACTAC,miR-142-3P50<2.2e-16
CTTTGCA,miR-52757<2.2e-16

a sex-related difference in CDH2 expression is an important factor affecting the prognosis of patients with ACC warrants further investigation. The transcript levels of CDH2 and CDH13 in patients with ACC aged >65 years were significantly lower than those in patients aged <65 years. Does this indicate an age advantage in the survival rate of patients with ACC? In this context, it is pertinent to mention that the survival rate of children with ACC who underwent surgery was lower than that of adults with ACC.17 Next, we attempted to explain the abnormalities in CDH2 and CDH13 expression through genetic alterations in patients. The expression of CDH2 and CDH13 was altered in 9% and 15% of patients with ACC, respectively. Abnormal expression of CDH2 and CDH13 caused by genetic changes may also be an important factor. DNA methylation affects the abnormal expression of CDH2 and CDH13 in cancer patients.18,19 However, this hypothesis warrants further investigation. These results suggest that CDH2 and CDH13 may serve as potential therapeutic and prognostic markers in patients with ACC.

CDH2, CDH13, and their ANGs are linked to a complex interaction network through coexpression and physical and genetic interactions. The molecular functions of CDH2 and its ANGs mainly include histone deacetylase binding, monoa- tomic ion transmembrane transporter activity, ATP hydroly- sis, protein kinase binding, and calcium ion binding. This shows that CDH2 and its ANGs may affect gene expression, cell cycle, and energy metabolism by regulating histone acet- ylation, protease activity, and ion channels, ultimately affecting tumor proliferation, differentiation, and metastasis. The mole- cular functions of CDH13 and its ANGs in patients with ACC include integrin binding, transcription corepressor activity, ATP-dependent activity, amide binding, and phospholipid binding. Thus, CDH13 and its ANGs may regulate the prolif- eration, invasion, migration, and angiogenesis of cancer cells by affecting integrin function, gene transcription, ability meta- bolism, and amide and phospholipid metabolism. Taken together, the functions involving CDH2, CDH13, and their ANGs may be involved in the occurrence and progression of ACC. Therefore, the regulation of these genes may be a potential treatment strategy for ACC.

Mining miRNA and kinase targets of key genes is an impor- tant breakthrough in ACC treatment. We found that miR-331, miR-486, miR-24, miR-101, miR-142-3P, and miR-527 are targets of CDH2 and CDH13 in patients with ACC. MiR-331, miR-24, miR-101, and miR-527 are associated with tumor cell proliferation, migration, invasion, and drug resistance, and may, therefore, be promising targets for cancer therapy.2 However, their relationship with ACC has not yet been reported. Furthermore, miR-486-3p may inhibit ACC cell pro- liferation by reducing the production of fatty acid synthases and fatty acids.23 Our results also indicate that miR-101, miR- 142-3P, and miR-527 are targets of CDH13 in patients with ACC. In a previous study, we showed that miR-142-3P might be an important regulatory target in ACC.24 We investigated the kinase targets of CDH2 and CDH13 in patients with ACC. We found that DYRK1B, LYN, NLK, TTK, CDK2, and CHEK1 were the kinase targets of CDH2 and CDH13. DYRK1B is a serine/threonine kinase involved in tumor progression and cell proliferation. Silencing or inactivation of DYRK1B may be a potential therapeutic strategy in cancer.25 Overexpression of LYN promotes the proliferation, migration, and invasion of cervical cancer cells by activating the IL-6/STAT3 pathway. Thus, it could be used as a novel target for the treatment of cervical cancer.26 NLK is a key regulator in many cancers. Lentivirus-mediated NLK knockout inhibited the growth and metastasis of small cell lung cancer; therefore it can be used as a potential target for the treatment of small cell lung cancer.27 However, its role in ACC has not yet been clarified. Furthermore, high expression of TTK, CDK2, and CHEK1 has been reported in ACC, which may play an impor- tant role in ACC progression and serve as potential biomarkers for future diagnosis and treatment.28-30 In summary, these miRNAs and kinases may serve as potential therapeutic targets for ACC.

We explored the correlation between the differentially expressed genes and CDH2 and CDH13 expression in patients with ACC. The expression of 4,824 and 2,748 genes was cor- related with CDH2 and CDH13 expression, respectively. Among these, VSNL1, TCF7, RASL10B, COL4A1, ANGPT2, and ESAM were the top six genes whose expression was posi- tively correlated with the expression of CDH2 and CDH13. Therefore, targeting these genes may provide additional ther- apeutic options for ACC. Immune infiltration is closely asso- ciated with tumor progression and prognosis.31 Cancer immunotherapy has led to significant advances in the treat- ment of multiple cancers. As expected, the expression levels of CDH13 in patients with ACC were positively correlated with immune cell infiltration. Targeting CDH13 or its related reg- ulatory targets may be a feasible strategy for improving the immune microenvironment in patients with ACC. We also

Table 4. The top three kinase target of CDH2 and CDH13 in ACC (LinkedOmics).
GeneKinase targetDescriptionLeading edge numberP-value
CDH2Kinase_DYRK1Bdual specificity tyrosine phosphorylation regulated kinase 1B2<2.2e-16
Kinase_LYNLYN proto-oncogene, Src family tyrosine kinase24<2.2e-16
Kinase_NLKnemo like kinase5<2.2e-16
CDH13Kinase_TTKTTK protein kinase9<2.2e-16
Kinase_CDK2cyclin dependent kinase 2102<2.2e-16
Kinase_CHEK1checkpoint kinase 142<2.2e-16
Figure 3. Genes differentially expressed in correlation with CDH2 and CDH13 expression in adrenocortical carcinoma (ACC) (obtained using LinkedOmics). (a and d) the Pearson test was used to analyze correlations between CDH2, CDH13, and genes differentially expressed in ACC, respectively; (b, c, e, and f) heat maps showing genes positively and negatively correlated with CDH2 and CDH13 in ACC, respectively (top 50 genes); the scatter plot shows Pearson correlation of CDH2 and CDH13 expression with expression of VSNL1 (g), TCF7 (h), RASL10B (i), COL4A1 (j), ANGPT2 (k), and ESAM (l) in ACC; red and blue indicate positively and negatively correlated genes, respectively.

a

CDH2 Association Result

b

CDH2

c

CDH2

1

CDH2

BRASIL 900

00

-log10(pvalue)

CO

PHOOLS WNT4 GYLTLID

Z-Score Group

Z-Score Group

-3

2

-3

2

1

0

1

0

0

A

0

*

MERTR

-1

<3

1

2

-1

<3

1 2

CV

CAOTS

A

0

THPT

GALNT14 GALNT15

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

2.0

HACIÔNG

LOC872550

Pearson Correlation Coefficient (Pearson test)

d

CDH13 Association Result

e

CDH13

f

CDH13

CDH13

GOL4AZ

10

SPOVE

EPLEBPS

-log10(pvalue)

2

Z-Score Group >3

Z-Score Group

2

-3

6

84

M

0

-1

2 4

1

0

<3

-1 <3

2 4

4

2

0

-1.5

-1.0

-0.5

0.0

0.5

1.0

1.5

2.0

Pearson Correlation Coefficient (Pearson test)

Pearson-Correlation:0.6735 P-value:1.043e-11

h

Pearson-Correlation:0.6491 P-value:9.77e-11

. i

Pearson-Correlation:0.6475 P-value:1.128e-10 Sample Size:(N=79)

Sample Size:(N=79)

Sample Size:(N=79)

CDH2

1

CDH2

12

CDH2

28

~

10

10

0

0

00

20

VSNL1

TCF7

RASL10B

6

00

6

4

6

+

2

+

0

2

0

2

4

6

8

10

12

2

4

6

8

10

12

2

4

6

8

10

12

CDH2

CDH2

CDH2

j

Pearson-Correlation:0.7066 P-value:3.436e-13 Sample Size:(N=79)

k

Pearson-Correlation:0.693 P-value:1.478e-12

1

Pearson-Correlation:0.6546 P-value:5.997e-11

00

Sample Size:(N=79)

Sample Size:(N=79)

CDH13

CDH13

CDH13

16

12

%

08

0

COL4A1

14

ANGPT2

ESAM

0

12

co

8

0

CO

+

00

-

5

6

7

8

9

10

5

6

7

8

9

10

5

6

7

8

9

10

CDH13

CDH13

CDH13

Figure 4. The correlation between CDH13 expression and immune cell infiltration and anti-PD1/CTLA-4/PD-L1 immunotherapy in adrenocortical carcinoma (ACC). (a) The correlation between CDH13 expression and immune cell infiltration levels in patients with ACC (TIMER); (b) the cumulative survival curve of CDH13 and immune cell infiltration in patients with ACC (TIMER); (c) heat maps showing the correlation between CDH13 and immune cell infiltration in ACC (BEST); (d-f) the correlation between CDH13 expression and immune score in patients with ACC (BEST); (g and i) boxplot showing the correlation between CDH13 expression and anti-PD1/CTLA-4/PD-L1 immunotherapy in ACC (BEST).

a

CDH13

CDH13 Expression Level (log2 TPM)

Purity

B Cell

CD8+ T Cell

CD4+ T Cell

Macrophage

Neutrophil

Dendritic Cell

6

cor = - 0.009

p = 9.39e-01

partial.cor = 0.363

p = 1.58e-03

partial.cor = 0.099

p = 4.06e-01

partial.cor = 0.301

p = 9.76e-03

partial.cor = 0.236

p = 4.41e-02

partial.cor = 0.232

p = 4.85e-02

partial.cor = 0.408

p = 3.37e-04

4-

..

2

ACC

0-

2

0.2

0.4

0.6

0.8

1.0

0.11

0.12

0.13

0.20

0.25

0.30

0.35 0.07

0.09

0.11

0.13

0.15

Infiltration Level

0.08

0.12

0.16

0.12

0.14

0.16

0.18

0.49

0.50

0.51

0.52

0.53

b

CDH13

B Cell

CD8+ T Cell

CD4+ T Cell

Macrophage

Neutrophil

Dendritic Cell

CDH13

1.0

Log-rank P = 0.396

Log-rank P = 0.05

Log-rank P = 0.724

Log-rank P = 0.175

Log-rank P = 0.475

Log-rank P = 0.601

Log-rank P = 0.04

Cumulative Survival

0.8

OS

0.6-

Expression

Expression Low

Expression Low

Expression Low

Expression

Expression Low

Expression Low

0.4

Low

High

High

High

High

High

High

High

0

CDH13

50

100

150

0

50

100

150

0

50

100

150

0

50

100

150

0

Time to Follow-Up (months)

50

100

150

0

50

100

150

0

50

100

150

C

507

ImmunsScore

d

Endothotel cells

GSE143383

e

GSE76019

f

GSE90713

Neutrophils

Cytotoxic mrhocytes

Cor = 0.355; Pval = 7e-03

Cor = 0.361; Pval = 3.7e-02

Cor = 0.411; Pval = 1.5e-03

3

Macrophage

2

StromalScore_estimate

2

StromalScore_estimate

2

StromalScore_estimate

Macrophages_MO

Dendrise_cols delivales

Toets folicular neiper

T_calls_CD4 memory mating

1

1

1

-

T_cols_CD4_memory_activened T_00“‘S Game maquiatory (Treges

0

Mast_cols resting

0

0

-1

Monocytes

T_Ous GD4

-1

-1

Macrophages_M1

-2

Macrophages_MO

Dendrisa_colis activated T_cells_CO4_naive

-2

1

0

1

2

-2

T_cells_gamma deita

-1

CDH13 Expression (z-score)

0

1

CDH13 Expression (z-score)

2

-3

-2

-1

0

1

2

T_cul_CD4_THE Og resing

CDH13 Expression (z-score)

T_bills_CD4 memory Foama

& o

Gao cohort 2018 Anti-PD-1/CTLA-4

h

Macrophages Mi

Macrophages M2

Kim cohort 2019 Anti-PD-1/PD-L1

1 IMvigor210 cohort 2018 Anti-PD-L1

T_ouls_GDE

T-test, p = 0.00033

T-test, p = 0.015

Wilcoxon, p = 0.032

Skeletal muscle

Hobalocytes

2

2

Smy Apracylas

CDH13 Expression (z-score)

CDH13 Expression (z-score)

CDH13 Expression (z-score)

2

Epithelial cells

Preadipocytes

CO4 ._ memory Tons

1

1

Class-switched_memory_8-cell

Macrophages_M

0

0

Melanocytes

Monocytes

0

CDA+_Tem

Macrophages

CO4.

Sabocytes

-1

Memory_@-calix

-1

CD4 ._ naive_

Eosino SOC

Keratinocytes

-2

CD4_1001S

-2

QSE19775

GEE143383

TOGALAGG

08E10927

NR

R

NR

R

NR

R

Response

Response

Response

found that high levels of CD8+ T-cell infiltration can possibly prolong the survival of patients with ACC. However, our results show that CDH13 expression is not related to the infiltration of CD8+ T cells. CDH13 and its ANGs can also activate T cells. These findings provide new avenues for ACC immunotherapy using CD8+ T cells. Furthermore, we found that the expression of CDH13 in patients with ACC who were

administered anti-PD1/CTLA-4/PD-L1 was strongly downre- gulated. Thus, patients with ACC who are treated with anti- PD1/CTLA-4/PD-L1 antibodies may have a better prognosis. However, the role of immunotherapy in ACC is limited.32 Studies have shown that the RTK signaling pathway inhibitor, foretinib, and the HSP90 inhibitor, elesclomol, have good antitumor effects and are safe.33,34 However, many tyrosine

Figure 5. IC50 evaluation of foretinib and elesclomol in different tissue types of cancer. (a and f) heat maps showing CDH2 and CDH13 low expression indicates resistance drugs ranking, respectively (BEST); (b) IC50 values of foretinib for the different cell lines (genomics of drug sensitivity in Cancer); (c) area under the curve (AUC) values of foretinib for the different cell lines (genomics of drug sensitivity in Cancer); (d) IC50 values of foretinib for the SW13 cell line (genomics of drug sensitivity in Cancer); (e) AUC values of foretinib for the SW13cell line (genomics of drug sensitivity in Cancer); (g) IC50 values of elesclomol for the different cell lines (genomics of drug sensitivity in Cancer); (h) AUC values of elesclomol for the different cell lines (genomics of drug sensitivity in Cancer); (i) IC50 values of elesclomol for the SW13 cell line (genomics of drug sensitivity in Cancer); (j) AUC values of elesclomol for the SW13 cell line (genomics of drug sensitivity in cancer).

a

b

Cell line IC50 values (Foretinib)

C Cell line AUC values (Foretinib)

High expression indicates sensitivity

Cisplatin_1005

1.0

104

TW 37_1149

Number of cell lines screened: 953

Number of cell lines screened: 953

Maximum AUC: 0.980

Thapsigargin_180

Maximum IC50 (uM): 3.12e3

Minimum AUC: 0.0177

SB52334_304

10ª

Geometric mean (uM): 2.48

0.8

MetAP2 Inhibitor, A832234_410

Minimum IC50 (uM): 0.00285

MCT1_6447_1436

102

AZD4547_1135

IC50 (micromolar)

Min screening concentration (uM): 0.0100

Max screening concentration (uM): 10 6

0.6

QS11_151

10

-…- max cone

AZD6738_1394

AUC

AZD7762_1402

Olaparib_1017

10

0.4

AZ20_1184

YM201636_310

10-

Methotrexate_1008

0.2

rTRAIL_1261

10-2

min conc

Foretinib_308

Correlation

TCGA_ACC GSE90713 GSE143383

GSE19775

1

0.5

10-3

0.0

0

ranked by sensitivity

ranked by sensitivity

-0.5

-1

d

e

Cell line (SW13) IC50 values (Foretinib)

Cell line (SW13) AUC values (Foretinib)

1.0

104

IC50 (uM): 3.25

AUC: 0.783

103

0.8

102

IC50 (micromolar)

0.6

10

max conc

AUC

100

0.4

10

0.2

10

min conc

103

0.0

ranked by sensitivity

ranked by sensitivity

09

h

f

Cell line IC50 values (Elesclomol)

Cell line AUC values (Elesclomol)

102

1.0

Number of cell lines screened: 921 Maximum IC50 (uM): 10.3

Number of cell lines screened: 921

Maximum AUC: 0.997

AZD6482_1066

101

Geometric mean (uM): 0.0486

Minimum IC50 (uM): 0.000231

Minimum AUC: 0.0209

0.8

AZD6482_156

Min screening concentration (uM): 0.000781

PLX-4720_1371

100

Max screening concentration (uM): 0 200

IAP_5620_1428

0.6

Sepantronium bromide_268

max conc

AZD1332_1463

10-

AUC

JAK3_7406_1434

0,4

High expression indicates sensitivity

IC50 (micromolar)

Cisplatin_1496

TW 37_1149

10ª

AZD4547_1135

FGFR_3831_1422

10º

0.2

IGFR_3801_1430

-min cono

BX795_1037

Talazoparib_1259

10

0.0

Cisplatin_1005

FEN1_3940_1419

ranked by sensitivity

CHIR-99021_154

1

j

Rucaparib_1175

Docetaxel_1007

Cell line (SW13) IC50 values (Elesclomol)

Cell line (SW13) AUC values (Elesclomol)

PARP_9495_1458

1.0

Piperlongumine_1243

102

AUC: 0.422

Alisertib_431

IC50 (uM): 0.00763

LIMK1 inhibitor BMS4_406

101

0.8

Doramapimod_1042

IOX2_1230

Olaparib_1495

IC50 (micromolar)

100

AZD4547_1497

0.6

RO-3306_1052

max conc

AUC

LDN-193189_478

10-

Elesclomol_1031

0.4

GSE19775

GSE33371

GSE10927

GSE12368

TCGA_ACC

GSE19750

GSE76021

GSE76019

GSE143383

GSE90713

Correlation

1

10ª

0.5

0

0.2

-0.5

104

-min conc

-1

104

0.0

ranked by sensitivity

ranked by sensitivity

kinase inhibitors against ACC (sunitinib, cabozantinib, and linsitinib) have been evaluated and have failed to obtain good results,35 but the effect of foretinib on ACC remains unclear. We evaluated the inhibitory effects of foretinib and elesclomol on SW13 (while SW13 may no longer be considered the ACC model, it was the only one analyzed owing to a lack of available information on H295R in the database). Foretinib and elesclo- mol exhibited broad-spectrum inhibitory effects on cancer cell lines. Foretinib and elesclomol may exert strong inhibitory effects on SW13 cells by inhibiting the expression of CDH2 and CDH13. Therefore, these drugs may be effective for the treatment of ACC. We identified the roles of CDH2 and CDH13 in ACC using bioinformatics methods. However, further validation through in vitro and ex vivo experiments is necessary to confirm their relationship.

In summary, our results provide insights into the expression, gene regulatory network, prognostic value, therapeutic targets, and drugs against CDH2 and CDH13 in patients with ACC. Our findings provide a better under- standing of the pathogenesis of ACC and could aid in devising effective treatment strategies. CDH2 and CDH13 may be potential prognostic and therapeutic targets of ACC.

Materials and methods

GEPIA

We used GEPIA (http://gepia.cancer-pku.cn/index.html) to analyze the relationships between gene expression, tumor pathological stages, and prognosis. The screening criteria were as follows: (1) genes: CDH2 and CDH13; (2) dataset: ACC; and (3) 77 patients; threshold-setting conditions: P-value cutoff = 0.05. The Student’s t-test was used to ana- lyze the expression of CDH2 and CDH13 in ACC. Kaplan - Meier curves were used to analyze the prognosis of patients with ACC.24

UALCAN

UALCAN (http://ualcan.path.uab.edu/analysis.html) is a comprehensive, user-friendly, and interactive web resource for mining and analysis of cancer data, mainly from The Cancer Genome Atlas (TCGA) database. We used UALCAN to analyze the expression of CDH2 and CDH13 in ACC. The “Expression Analysis” module of the UALCAN database was used to analyze TCGA gene expres- sion data; the screening criteria were set as follows: (1) genes: CDH2 and CDH13; (2) dataset: ACC; (3) 79 ACC patients (31 male and 48 female); threshold setting condi- tions: P-value cutoff = 0.05. The Student’s t-test was used for comparative analysis.24

BEST

BEST (https://rookieutopia.com/app_direct/BEST/) provides a curated database and innovative analytical pipelines to explore cancer biomarkers at a high resolution. Protein expres- sion, immune cell infiltration, candidate agents, and

immunotherapy targeting CDH2 and CDH13 in ACC were analyzed using BEST. The “Clinical association,” “Cell infiltra- tion,” “Immunotherapy,” and “Candidate agents” modules of the BEST database were used to analyze gene expression omnibus and TCGA gene expression data using the following screening criteria: (1) genes: CDH2 and CDH13; (2) dataset: ACC (10 datasets and 508 patients).24

cBioPortal

cBioPortal (http://cbioportal.org) is an online database used for tumor gene mutation analysis. We used cBioPortal to analyze alterations in CDH2, CDH13, and the top 50 ANGs. A total of 75 ACC samples were analyzed, and z-scores for mRNA expression relative to all samples (log RNA Seq V2 RSEM) were obtained using a z-score threshold of ± 2.0.24

STRING and GeneMANIA

STRING (https://string-db.org/cgi/input.pl.) and GeneMANIA (http://www.genemania.org.) are online data- bases used for analyzing gene - protein and PPI networks. STRING was used to build a low-confidence level (0.150) PPI network and screen criteria for species defined as humans. GeneMANIA was used to explore the functions of CDH2, CDH13, and their top 50 ANGs.24

Metascape

Metascape (https://metascape.org) is an online database used to analyze the functions and signaling pathways of genes and proteins. We used Metascape to analyze the functions and signaling pathways of CDH2, CDH13, and their top 50 ANGs.24

LinkedOmics

LinkedOmics (http://www.linkedomics.org/) is a public online platform for analyzing correlations between differentially expressed genes related to tumor target genes and for predict- ing miRNA and kinase targets. It was used to identify kinase targets, miRNA targets, and differentially expressed genes related to CDH2 and CDH13.24

TIMER

TIMER (https://cistome.shinyapps.io/timer/) is an online database used to analyze the relationship between tumor genes and infiltrating immune cells. We used it to analyze the correlation between CDH2 and CDH13 expression and immune cell infiltration.

Genomics of drug sensitivity in cancer analysis

Genomics of drug sensitivity in cancer (http://www. cancerRxgene.org) is a specialized public database for obtain- ing information on potential anticancer drugs. We used this database to identify drugs targeting CDH2 and CDH13 and to predict their anti-ACC activity.24

Disclosure statement

No potential conflict of interest was reported by the author(s).

Funding

This research was funded by postdoctoral Foundation of Guangdong Medical University [4SG22292G] and National Natural Science Foundation of China Youth Science Foundation Program [31101639].

ORCID

Yongli Situ [D http://orcid.org/0000-0003-2244-115X

Data availability statement

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

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