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A422V

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
AlanineValine at position 422 · TM3 (402-422), helical transmembrane · WFS1 (Wolframin)

Alanine → Valine at position 422 inside TM3. ClinVar Conflicting including monogenic diabetes + WFS1 spectrum. AlphaMissense 0.23 (below threshold) — AM under-call. DynaMut2 ΔΔG +0.31 STABILISING.

Interactive 3D Structure

Wild-type reference
Wild-type A422 — hydrogen bond to S418
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DynaMut2 mutant · A422V
Mutant V422 — hydrophobic contact to L347 lost
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Bond changes · DynaMut2 interaction analysis

0 lost3 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondS418S418Preserved
Hydrogen bondF419Gained
Polar contactP346Gained
Polar contactS418S418Preserved
Polar contactK424K424Preserved
Van der WaalsS418S418Preserved
HydrophobicP346P346Preserved
HydrophobicL347L347Preserved
HydrophobicF350Gained
HydrophobicI427I427Preserved

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
0.31kcal/mol
Stabilising — mild
AlphaMissense
0.234
LBen
AlphaFold pLDDT
87
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWFS1-Related Spectrum Disorders; Monogenic diabetes
InheritanceMulti-phenotype.
Population frequency (gnomAD v4)Low frequency · AF 0.042%
cDNA changec.1265C>T
ClinVar accessionVCV000393388
Last evaluated2026/01/23 00:00

Observed in the general population.

Structural Context

Position 422 at TM3 end. Neighbors: SER423 (2.4 Å), ILE421 (2.5 Å), SER418 (3.7 Å — TM2-TM3 interface, same S418 as F350I).

A422V at the TM3 lumenal end. Conservative volume increase + stabilising ΔΔG. AM 0.23 under-call; multi-phenotype confirms pathogenicity. The S418 cross-helix contact is structurally significant.

Amino-acid chemistry
Alanine (A) → Valine (V) — small replaced by branched aliphatic.
Position in the protein
TM3 (residues 402–422) · position 422 at TM3 end (pLDDT 87).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). ΔΔG +0.31. AlphaMissense 0.23 below threshold but multi-phenotype confirms pathogenicity.

Mechanism: TM3-TM2 interface perturbation at S418. Therapeutic: same target as F350I, V412L, V412A.

Why this matters

A422V continues TM3-TM2 interface convergence.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A422V 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 A422V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane402422 · Helical