STRC

Chr 15AR

stereocilin

Also known as: DFNB16

The protein is essential for formation of horizontal top connectors between outer hair cell stereocilia in the inner ear, which are critical for mechanoreception of sound waves. Mutations cause autosomal recessive non-syndromic deafness (DFNB16), typically presenting as congenital hearing loss. This gene shows extremely low constraint against loss-of-function variants in the general population and follows autosomal recessive inheritance.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismARLOEUF 0.881 OMIM phenotype
Clinical SummarySTRC
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Gene-Disease Validity (ClinGen)
nonsyndromic genetic hearing loss · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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GeneReview available — STRC
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.88LOEUF
pLI 0.000
Z-score 2.23
OE 0.65 (0.490.88)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.27Z-score
OE missense 0.83 (0.760.91)
374 obs / 449.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.65 (0.490.88)
00.351.4
Missense OE0.83 (0.760.91)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 31 / 47.6Missense obs/exp: 374 / 449.7Syn Z: 1.71
DN
0.5968th %ile
GOF
0.6833th %ile
LOF
0.3068th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

STRC · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC