SLC26A5

Chr 7

solute carrier family 26 member 5

Also known as: DFNB61, PRES

This gene encodes a member of the SLC26A/SulP transporter family. The protein functions as a molecular motor in motile outer hair cells (OHCs) of the cochlea, inducing changes in cell length that act to amplify sound levels. The transmembrane protein is an incomplete anion transporter, and does not allow anions to cross the cell membrane but instead undergoes a conformational change in response to changes in intracellular Cl- levels that results in a change in cell length. The protein functions at microsecond rates, which is several orders of magnitude faster than conventional molecular motor proteins. Mutations in this gene are potential candidates for causing neurosensory deafness. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2009]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDeafness, autosomal recessive, 61

Clinical highlights

Gene-disease validity (ClinGen)
nonsyndromic genetic hearing loss · ARLimitednot for standalone diagnostic reporting
0
Active trials
8
Pubs (1 yr)
P/LP submissions
P/LP missense
0.72
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — SLC26A5
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.72LOEUF
pLI 0.000
Z-score 2.88
OE 0.49 (0.340.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.90Z-score
OE missense 0.87 (0.800.95)
355 obs / 406.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.49 (0.340.72)
00.351.4
Missense OE?0.87 (0.800.95)
00.61.4
Synonymous OE?0.88
01.21.6
LoF obs/exp: 18 / 36.9Missense obs/exp: 355 / 406.1Syn Z: 1.14

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.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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