Adult treatment window for STRC
Window status. Open in adults for STRC, unlike most HC genes. Reason. Stereocilin is continuously expressed to maintain horizontal top connectors and tectorial attachment in mature OHCs. LoF patients have living OHCs with defective connector architecture — not dead/missing HCs. Restoring STRC protein should restore function.
Evidence base.
- 2021 Tarchini et al: neonatal P1 rescue in mouse (ABR -50 dB improvement)
- 2026 Iranfar et al: extended window to P30 (equivalent to pre-adolescent human)
- 2026 Tsai SLC26A4: postnatal rescue in different DFNB gene, establishes principle
- Misha at 7yo: within plausible human window per mouse extrapolation + OHC persistence.
Contrast.
- TMC1: HC death by P14 → window closes
- OTOF: IHC function, synaptopathy, window limited
- STRC: OHCs alive, architecture broken → window open
Connections
[about]STRC Gene[supports]index therapy strategy[source]2021-12-holt-strc-dual-aav-science-advances[source]2026-01-iranfar-dual-aav-strc-ctm[source]2026-04-16-tsai-slc26a4-postnatal-gene-therapy