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Gene profile

GJB2

HGNC:4284 · SaudiVarKB evidence summary derived from retained literature mentions.

11Gene mentions
11Publications
5Linked variants
1Associated phenotypes

Associated phenotypes

Co-mentioned in GJB2 publications
PhenotypeIdentifierArticlesMentions
hearing lossHP:000036599

Linked variants

Variants normalized to GJB2
VariantHGVS / rsIDArticlesMentions
A7445G11
p.Cys169Tyrp.Cys169Tyr11
p.Glu57*p.Glu57*11
p.Arg1792Hisp.Arg1792His11
c.35delGc.35delG11

Population context

Reported in the same publications
Country / regionGroupCohortArticles
Not specified2
Saudi Arabia1
Not specified401
Saudi Arabia501
Not specified441
Saudi Arabia1001
Saudi Arabia3661
Saudi Arabia331
Saudi Arabia1091

Supporting publications

11 records
  1. 2025Consanguinity and rare monogenic systemic autoinflammatory disorders: implications for prevalence and genetic variability.Pediatric rheumatology online journalPubMed ↗
  2. 2024Testing for genetic and viral etiologies in congenital hearing loss based on a survey of cochlear implant centers: proposed HEARRING group consensus and future directions.Acta oto-laryngologicaPubMed ↗
  3. 2021Whole exome sequencing, in silico and functional studies confirm the association of the GJB2 mutation p.Cys169Tyr with deafness and suggest a role for the TMEM59 gene in the hearing process.Saudi journal of biological sciencesPubMed ↗
  4. 2020Identification of TMC1 as a relatively common cause for nonsyndromic hearing loss in the Saudi population.American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric GeneticsPubMed ↗
  5. 2019Sequence variants in genes causing nonsyndromic hearing loss in a Pakistani cohort.Molecular genetics & genomic medicinePubMed ↗
  6. 2018Recurrent variants in OTOF are significant contributors to prelingual nonsydromic hearing loss in Saudi patients.Genetics in medicine : official journal of the American College of Medical GeneticsPubMed ↗
  7. 2017Unique autosomal recessive variant of palmoplantar keratoderma associated with hearing loss not caused by known mutations.Anais brasileiros de dermatologiaPubMed ↗
  8. 2016Utilization of amplicon-based targeted sequencing panel for the massively parallel sequencing of sporadic hearing impairment patients from Saudi Arabia.BMC medical geneticsPubMed ↗
  9. 2013Homozygosity mapping identifies a novel GIPC3 mutation causing congenital nonsyndromic hearing loss in a Saudi family.GenePubMed ↗
  10. 2010Spectrum of GJB2 mutations in a cohort of nonsyndromic hearing loss cases from the Kingdom of Saudi Arabia.Genetic testing and molecular biomarkersPubMed ↗
  11. 2010High homogeneity in auditory outcome of pediatric CI-patients with mutations in Gap-Junction-Protein Beta2.International journal of pediatric otorhinolaryngologyPubMed ↗