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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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GJB4
gap junction protein beta 4
Chromosome 1 Β· 1p34.3
NCBI Gene: 127534Ensembl: ENSG00000189433.8HGNC: HGNC:4286UniProt: Q9NTQ9
30PubMed Papers
21Diseases
0Drugs
5Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
cell junctionprotein bindinggap junction channel activitycell-cell signalingerythrokeratodermia variabilisalcohol drinkingliver diseasemalignant renal pelvis neoplasm
✦AI Summary

GJB4 encodes gap junction protein beta 4 (connexin 30.3), a structural component of gap junctions that form dodecameric channels connecting adjacent cell cytoplasm 1. These channels permit diffusion of small molecules and ions between cells through intercellular communication 1. GJB4 variants cause erythrokeratodermia variabilis et progressiva (EKVP), a rare hereditary skin disorder characterized by hyperkeratotic plaques and erythematous patches 2. GJB4 mutations elicit multiple molecular defects including impaired gap junction formation, dysregulated hemichannel function, and altered channel kinetics 2. Specific mutations in transmembrane domains (T85P, F137L) affect voltage gating and channel closure kinetics, while amino-terminal mutations may interfere with connexin selectivity 3. Beyond dermatology, GJB4 shows unexpected roles in malignancy. Upregulated GJB4 expression in pancreatic cancer correlates with poor prognosis and promotes tumor growth through MET-AKT pathway activation, with GJB4 knockdown inducing ferroptosis and suppressing metastasis 45. Similarly, in lung cancer, elevated GJB4 promotes metastasis and chemoresistance via Src activation and serves as a prognostic biomarker 6. Conversely, GJB4 overexpression in pancreatic islet cells impairs insulin secretion and cell proliferation, suggesting involvement in type 2 diabetes pathogenesis 7.

Sources cited
1
GJB4 variants cause EKVP through multiple molecular defects including impaired gap junction formation and dysregulated hemichannel function
PMID: 39513663
2
GJB4 encodes connexin 30.3, a gap junction component; mutations cause EKVP with hyperkeratotic plaques and erythematous patches
PMID: 26945536
3
GJB4 mutations in transmembrane domains affect voltage gating and channel closure; amino-terminal mutations affect connexin selectivity
PMID: 12648223
4
GJB4 upregulation in pancreatic cancer promotes tumor growth; GJB4 knockdown inhibits proliferation and promotes ferroptosis
PMID: 40575171
5
GJB4 upregulation in pancreatic cancer activates MET-AKT pathway; GJB4 knockdown causes G0/G1 arrest and suppresses metastasis
PMID: 38342100
6
GJB4 overexpression in lung cancer promotes metastasis and chemoresistance via Src activation and serves as prognostic biomarker
PMID: 30177841
7
GJB4 overexpression in pancreatic islet cells inhibits proliferation and insulin secretion, implicating it in type 2 diabetes
PMID: 32565358
Disease Associationsβ“˜21
erythrokeratodermia variabilisOpen Targets
0.77Strong
alcohol drinkingOpen Targets
0.22Weak
liver diseaseOpen Targets
0.20Weak
malignant renal pelvis neoplasmOpen Targets
0.19Weak
genetic disorderOpen Targets
0.19Weak
gallbladder diseaseOpen Targets
0.19Weak
gestational diabetesOpen Targets
0.17Weak
poisoningOpen Targets
0.16Weak
hemolytic anemiaOpen Targets
0.15Weak
autosomal dominant nonsyndromic hearing loss 3AOpen Targets
0.12Weak
autosomal recessive nonsyndromic hearing loss 1AOpen Targets
0.12Weak
hearing loss, autosomal recessiveOpen Targets
0.12Weak
neoplasmOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
pachyonychia congenitaOpen Targets
0.07Suggestive
Leber congenital amaurosis 10Open Targets
0.05Suggestive
diabetes mellitusOpen Targets
0.04Suggestive
Testicular regression syndromeOpen Targets
0.04Suggestive
Subdural hemorrhageOpen Targets
0.04Suggestive
46,XX ovotesticular disorder of sex developmentOpen Targets
0.04Suggestive
Erythrokeratodermia variabilis et progressiva 2UniProt
Pathogenic Variants5
NM_153212.3(GJB4):c.409T>C (p.Phe137Leu)Likely pathogenic
Erythrokeratodermia variabilis et progressiva 2|not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 137
NM_153212.3(GJB4):c.253A>C (p.Thr85Pro)Pathogenic
Erythrokeratodermia variabilis et progressiva 2|not provided
β˜…β˜†β˜†β˜†2016β†’ Residue 85
NM_153212.3(GJB4):c.411C>A (p.Phe137Leu)Pathogenic
Erythrokeratodermia variabilis et progressiva 2
β˜†β˜†β˜†β˜†2003β†’ Residue 137
NM_153212.3(GJB4):c.35G>A (p.Gly12Asp)Pathogenic
Erythrokeratodermia variabilis et progressiva 2|not provided
β˜†β˜†β˜†β˜†2003β†’ Residue 12
NM_153212.3(GJB4):c.566T>A (p.Phe189Tyr)Pathogenic
Erythrokeratodermia variabilis et progressiva 2
β˜†β˜†β˜†β˜†2003β†’ Residue 189
View on ClinVar β†—
Related Genes
GJA1Protein interaction97%GJA8Protein interaction84%GJA3Protein interaction75%GJA5Protein interaction74%GJB1Protein interaction74%GJD3Protein interaction74%
Tissue Expression6 tissues
Brain
100%
Liver
77%
Lung
46%
Ovary
15%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
GJB4GJA1GJA8GJA3GJA5GJB1GJD3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q9NTQ9
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
1.92LoF Tolerant
pLIβ“˜
0.13Tolerant
Observed/Expected LoF1.65 [0.33–1.92]
RankingsWhere GJB4 stands among ~20K protein-coding genes
  • #11,929of 20,598
    Most Researched30
  • #3,621of 5,498
    Most Pathogenic Variants5
  • #17,415of 17,882
    Most Constrained (LOEUF)1.92
Genes detectedGJB4
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
The genetic and molecular basis of a connexin-linked skin disease.
PMID: 39513663
Biochem J Β· 2024
1.00
2
Erythrokeratodermia variabilis et progressiva.
PMID: 26945536
J Dermatol Β· 2016
0.90
3
Targeting GJB4 to inhibit tumor growth and induce ferroptosis in pancreatic cancer.
PMID: 40575171
Front Oncol Β· 2025
0.80
4
Gjb4 serves as a novel biomarker for lung cancer and promotes metastasis and chemoresistance via Src activation.
PMID: 30177841
Oncogene Β· 2019
0.70
5
PMID: 32524838
Exp Biol Med (Maywood) Β· 2020
0.60