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rs111033220

From SNPedia

Orientationplus
Stabilizedplus
Geno Mag Summary
(C;C) 0 common in clinvar
(C;T) 3 Carrier of a mutation for Pendred syndrome (hearing loss)
(T;T) 5 Pendred syndrome (hearing loss)
ReferenceGRCh38 38.1/141
Chromosome7
Position107690203
GeneSLC26A4
is asnp
is mentioned by
dbSNPrs111033220
dbSNP (classic)rs111033220
ClinGenrs111033220
ebirs111033220
HLIrs111033220
Exacrs111033220
Gnomadrs111033220
Varsomers111033220
LitVarrs111033220
Maprs111033220
PheGenIrs111033220
Biobankrs111033220
1000 genomesrs111033220
hgdprs111033220
ensemblrs111033220
geneviewrs111033220
scholarrs111033220
googlers111033220
pharmgkbrs111033220
gwascentralrs111033220
openSNPrs111033220
23andMers111033220
SNPshotrs111033220
SNPdbers111033220
MSV3drs111033220
GWAS Ctlgrs111033220
Max Magnitude5

rs111033220, also known as c.1229C>T, p.Thr410Met and T410M, represents a rare mutation in the SLC26A4 gene.

Inherited recessively, this variant is considered pathogenic for Pendred Syndrome, manifest as hearing loss and enlarged vestibular aqueduct syndrome.


ClinVar
Risk Rs111033220(T;T)
Alt Rs111033220(T;T)
Reference Rs111033220(C;C)
Significance Pathogenic
Disease Enlarged vestibular aqueduct syndrome Pendred's syndrome not provided
Variation info
Gene SLC26A4
CLNDBN Enlarged vestibular aqueduct syndrome Pendred's syndrome not provided
Reversed 0
HGVS NC_000007.13:g.107330648C>T
CLNSRC UniProtKB (protein)
CLNACC RCV000036430.2, RCV000268093.1,


[PMID 119323] Effect of mannitol on the traumatized spinal cord. Microangiography, blood flow patterns, and electrophysiology.


[PMID 9618167] Molecular analysis of the PDS gene in Pendred syndrome.


[PMID 10700480] Enlarged vestibular aqueduct: a radiological marker of pendred syndrome, and mutation of the PDS gene.


[PMID 11317356] Pendred syndrome, DFNB4, and PDS/SLC26A4 identification of eight novel mutations and possible genotype-phenotype correlations.


[PMID 11919333] Differential diagnosis between Pendred and pseudo-Pendred syndromes: clinical, radiologic, and molecular studies.


[PMID 15355436] Screening of SLC26A4 (PDS) gene in Pendred's syndrome: a large spectrum of mutations in France and phenotypic heterogeneity.