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A342T

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
AlanineThreonine at position 342 · TM2 (340-360), helical transmembrane · WFS1 (Wolframin)

Alanine → Threonine at position 342 inside TM2. ClinVar Conflicting including WFS1 spectrum + Wolfram. AlphaMissense 0.13 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.72.

Interactive 3D Structure

Wild-type reference
Wild-type A342 — hydrogen bond to I338
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DynaMut2 mutant · A342T
Mutant T342 — van der waals contact to P346 lost
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Bond changes · DynaMut2 interaction analysis

1 lost6 gained6 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondI338I338Preserved
Hydrogen bondD339D339Preserved
Hydrogen bondW867Gained
Polar contactI338I338Preserved
Polar contactD339D339Preserved
Polar contactF340Gained
Polar contactP346P346Preserved
Polar contactW867Gained
Van der WaalsD339Gained
Van der WaalsF340Gained
Van der WaalsP346Lost
Van der WaalsW867Gained
HydrophobicW867W867Preserved

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
-0.72kcal/mol
Destabilising — mild
AlphaMissense
0.131
LBen
AlphaFold pLDDT
73
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; Wolfram syndrome 1
InheritanceMulti-phenotype.
Population frequency (gnomAD v4)Low frequency · AF 0.020%
cDNA changec.1024G>A
ClinVar accessionVCV000130747
Last evaluated2024/08/28 00:00

Observed in the general population.

Structural Context

Position 342 at TM2 start. Neighbors: PHE343 (2.4 Å), PHE341 (2.5 Å — aromatic cluster start), TRP867 (3.6 Å — long-range to TM11 W867!). The W867 contact is structurally significant — TM2-TM11 cross-helix contact.

A342T introduces polarity into TM2 + perturbs TM2-TM11 cross-helix W867 contact. AM 0.13 under-call; multi-phenotype confirms.

Amino-acid chemistry
Alanine (A) → Threonine (T) — small replaced by polar hydroxyl.
Position in the protein
TM2 (residues 340–360) · position 342 near TM2 start (pLDDT 73).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.72. AlphaMissense 0.13 below threshold but multi-phenotype confirms.

Mechanism: polarity in TM2 + TM2-TM11 W867 cross-helix disruption. Therapeutic: TM2-TM11 interface.

Why this matters

A342T identifies a TM2-TM11 cross-helix contact at W867 — new interface target.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A342T 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 A342T PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane340360 · Helical