STRC (stereocilin gene)
Locus. 15q15.3, 29 exons, 6.2 kb CDS, 1809 aa protein. Product. Stereocilin — GPI-anchored OHC stereocilia protein; horizontal top connectors + tectorial attachment. Disease. Biallelic LoF → STRC Hearing Loss (DFNB16), mild-to-moderate sensorineural, ~2% of nonsyndromic HL, ~15% of mild-moderate. Pseudogene. STRCP1 sits ~100 kb centromeric, 97% identical; drives diagnostic false-positives and limits standard NGS. Dual-AAV splitting respects paralog homology risk.
key numbers
| Property | Value | Source |
|---|---|---|
| Length | 6231 bp CDS | DFNB16 Hearing Loss |
| Protein | 1809 aa | 2024-genereviews-strc-dfnb16 |
| Prevalence | ~2% NSHL | genereviews |
| Pseudogene identity | 97% | STRCP1 |
| AAV cap | ~4.7 kb single-vector | 2021-12-holt-strc-dual-aav |
Connections
[part-of]index[about]STRC Hearing Loss[see-also]DFNB16 Hearing Loss[source]2024-genereviews-strc-dfnb16