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Mini-STRC Hypothesis

NEW COMPUTATIONAL

Current STRC gene therapy requires two AAV vectors because the gene (5325 bp) exceeds the single-AAV packaging limit (~4400 bp usable). AlphaFold structural analysis suggests a single-vector approach may be possible.

The packaging problem

Full STRC cDNA 5325 bp
AAV limit (with promoter/ITR) ~4400 bp
Mini-STRC (predicted) 3984 bp

What AlphaFold reveals

AlphaFold predicts stereocilin's structure with varying confidence along the protein. The N-terminal region (residues 1-615) has very low confidence (pLDDT < 50), indicating it is likely intrinsically disordered with no stable 3D structure. The functional core starts around residue 616.

E1659
cut here
1 N-terminal (disordered) LRR domain C-terminal (functional core) 1775

Remove (447 aa, 1341 bp)

  • 23-114 N-terminal disordered (pLDDT 30.6)
  • 132-251 Disordered region (pLDDT 37.0)
  • 309-387 Disordered loops (pLDDT 38-47)
  • 449-485 Disordered loop (pLDDT 47.5)
  • 496-615 Large disordered region (pLDDT 31.1)

All regions have pLDDT < 50 (no stable structure predicted)

Keep (1328 aa, 3984 bp)

  • 1-22 Signal peptide (secretion)
  • 616-1074 LRR domain (protein interactions)
  • 1075-1775 C-terminal (tectorial membrane attachment)
  • 1659 Misha's variant position (preserved)
  • 8 glycosylation sites in functional core preserved

3984 bp fits in single AAV (<4400 bp limit)

Precedent: micro-dystrophin

This approach has proven precedent. The dystrophin gene (11,000 bp) was too large for any AAV. Researchers created "micro-dystrophin" by removing non-essential spectrin-like repeats, fitting it into a single AAV. This is now in Phase 3 clinical trials (Sarepta SRP-9001). The same principle: identify the structural core, remove disordered/redundant regions, preserve function. Nobody has tried this for STRC yet.

Important: This is a computational hypothesis based on AlphaFold structural predictions. It requires experimental validation: does mini-stereocilin fold correctly? Does it localize to stereocilia tips? Does it form horizontal top connectors and tectorial membrane attachments? These questions need wet-lab work. But the structural data strongly suggests the N-terminal region is dispensable, and a single-AAV mini-STRC approach deserves investigation.

AlphaFold 3 Experiments

6 JOBS

Systematic computational testing of the mini-STRC hypothesis and variant impact. 3D models rendered live from AlphaFold 3 CIF files. Drag to rotate, scroll to zoom.

Key finding

Mini-STRC (without N-terminal) achieves pTM 0.81, significantly better than full-length wildtype (pTM 0.63). The removed N-terminal region scores only pTM 0.27 with 38% disorder. Removing the disordered N-terminal produces a better-folding protein that fits in a single AAV vector.

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