SLC26A5

Chr 7AR

solute carrier family 26 member 5

Also known as: DFNB61, PRES

The protein functions as a voltage-sensitive molecular motor in cochlear outer hair cells, converting electrical signals into mechanical contractions at microsecond speeds to amplify sound. Mutations cause autosomal recessive nonsyndromic sensorineural hearing loss. This gene is not highly constrained against loss-of-function variants and has an established GeneReviews entry for clinical guidance.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismAR1 OMIM phenotype
Clinical SummarySLC26A5
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Gene-Disease Validity (ClinGen)
nonsyndromic genetic hearing loss · ARLimited

Limited evidence — not for standalone diagnostic reporting

Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.81top 10%
GOF
0.82top 10%
LOF
0.1895th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SLC26A5 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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