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A326V

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
AlanineValine at position 326 · TM1 (314-334), helical transmembrane · WFS1 (Wolframin)

Alanine → Valine at position 326 inside TM1. ClinVar Conflicting for Wolfram syndrome 1. AlphaMissense 0.29 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.03 kcal/mol (neutral). Third Atlas substitution at position 326 (with A326E, A326T).

Interactive 3D Structure

Wild-type reference
Wild-type A326 — hydrogen bond to H322
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DynaMut2 mutant · A326V
Mutant V326 — hydrophobic contact to F330 lost
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Bond changes · DynaMut2 interaction analysis

0 lost0 gained11 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondH322H322Preserved
Hydrogen bondH323H323Preserved
Hydrogen bondF329F329Preserved
Hydrogen bondF330F330Preserved
Polar contactH322H322Preserved
Polar contactH323H323Preserved
Polar contactI324I324Preserved
Polar contactF329F329Preserved
Polar contactF330F330Preserved
Van der WaalsI324I324Preserved
HydrophobicF330F330Preserved

Lost / gained / preserved interatomic contacts at the variant residue, from the DynaMut2 (Arpeggio) interaction analysis of the wild-type and energy-minimized mutant structures.

Computational Predictions

DynaMut2 ΔΔG
-0.03kcal/mol
Destabilising — mild
AlphaMissense
0.288
LBen
AlphaFold pLDDT
77
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0080%
cDNA changec.977C>T
ClinVar accessionVCV000592987
Last evaluated2025/11/27 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 326 same neighbors as A326E/A326T: ASN325 (2.5 Å), LEU327 (2.5 Å), HIS322 (3.6 Å — H323R neighbor cluster). The H322-H323-A326 microregion is a multi-variant hub in TM1.

A326V is the most conservative substitution at position 326 (with A326E charge introduction and A326T polar introduction). All three pathogenic — position 326 is structurally inflexible regardless of substitution chemistry.

Amino-acid chemistry
Alanine (A) → Valine (V) — small methyl replaced by branched aliphatic. Conservative volume increase.
Position in the protein
TM1 (residues 314–334) · position 326 (pLDDT 77). Same as A326E, A326T.

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). ΔΔG ≈ 0. AlphaMissense 0.29 below threshold but Wolfram 1 + dual-position substitutions confirm pathogenicity.

Therapeutic strategy: same TM1 H322-H323-A326 microregion as A326E, A326T, H323R.

Why this matters

A326V completes the THIRD substitution at position 326 (with A326E, A326T) — multi-substitution hotspot.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A326V PDF below and upload it to Wolfram Intelligence to generate therapeutic-strategy proposals — guanidinium mimetics, sigma-1 agonist docking, NAC thiol-capping. NAC is already on the bench-testing list.

Download A326V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane314334 · Helical
Natural variant326326 · in dbSNP:rs369795224