RareResearch.AI
← Back to atlas

A326T

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

Alanine → Threonine at position 326 inside TM1. ClinVar Conflicting including Wolfram + inborn diseases. AlphaMissense 0.13 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.79. Third substitution at position 326 (with A326E, A326V).

Interactive 3D Structure

Wild-type reference
Wild-type A326 — hydrogen bond to H322
Fullscreen ↗
DynaMut2 mutant · A326T
Mutant T326 — hydrogen bond contact to H323 lost
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

0 lost1 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 WaalsH322Gained
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
-0.79kcal/mol
Destabilising — mild
AlphaMissense
0.128
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 conditionsInborn genetic diseases; Wolfram syndrome 1
InheritanceMulti-phenotype.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0067%
cDNA changec.976G>A
ClinVar accessionVCV001584586
Last evaluated2026/02/01 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 326 same neighbors as A326E/A326V: ASN325 (2.5 Å), LEU327 (2.5 Å), HIS322 (3.6 Å).

A326T is the THIRD substitution at position 326 (with A326E charge, A326V volume). Position 326 is structurally inflexible regardless of substitution chemistry — three variants confirm this. AM 0.13 under-call; multi-phenotype confirms.

Amino-acid chemistry
Alanine (A) → Threonine (T) — small replaced by polar hydroxyl.
Position in the protein
TM1 (residues 314–334) · position 326 (pLDDT 77). Same as A326E, A326V.

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.79. AlphaMissense 0.13 below threshold but multi-phenotype + three-substitution position confirm pathogenicity.

Mechanism: polarity introduction at structurally critical TM1 position. Therapeutic: same TM1 microregion as A326E, A326V.

Why this matters

A326T completes the THIRD substitution at position 326 — three pathogenic variants establish position 326 as inflexible.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A326T 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 A326T PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

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