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T461I

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
ThreonineIsoleucine at position 461 · Connecting loop · WFS1 (Wolframin)

Threonine → Isoleucine at position 461 in a connecting loop. ClinVar Conflicting including Wolfram syndrome 1. AlphaMissense 0.30 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.30 kcal/mol. Adjacent to E462G (Atlas card).

Interactive 3D Structure

Wild-type reference
Wild-type T461 — hydrogen bond to R457
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DynaMut2 mutant · T461I
Mutant I461 — hydrogen bond contact to R457 lost
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Bond changes · DynaMut2 interaction analysis

1 lost6 gained12 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondR457R457Preserved
Hydrogen bondA458A458Preserved
Hydrogen bondT464T464Preserved
Hydrogen bondA465A465Preserved
Hydrogen bondL507Gained
Polar contactR457R457Preserved
Polar contactA458A458Preserved
Polar contactL459L459Preserved
Polar contactT464Gained
Polar contactA465A465Preserved
Van der WaalsA458A458Preserved
Van der WaalsL459L459Preserved
Van der WaalsA465A465Preserved
Van der WaalsY510Lost
Van der WaalsL511Gained
HydrophobicL507Gained
HydrophobicY510Y510Preserved
HydrophobicL511Gained
HydrophobicL514Gained

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
-0.30kcal/mol
Destabilising — mild
AlphaMissense
0.296
LBen
AlphaFold pLDDT
86
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 0.00014%
cDNA changec.1382C>T
ClinVar accessionVCV000229636
Last evaluated2015/12/31 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 461 sits in a connecting loop, immediately upstream of E462 (E462G Atlas card). Neighbors: GLU462 (2.5 Å — the E462G variant position!), ALA460 (2.5 Å), ALA458 (3.6 Å). The E462 contact at 2.5 Å places T461 in direct sequence contact with the E462G pathogenic variant.

The wild-type T461 hydroxyl likely H-bonds with E462's carboxylate, stabilizing the local loop geometry. Replacing T461 with isoleucine eliminates the H-bonding capacity; the E462 carboxylate loses its sequence-neighbor H-bond partner.

|ΔΔG| 0.30 + AM 0.30 below threshold + Wolfram syndrome 1 confirm pathogenicity. The mechanism is loss of T461-E462 H-bond — perturbing the same loop microregion that E462G disrupts.

Amino-acid chemistry
Threonine (T) → Isoleucine (I) — small polar hydroxyl replaced by branched aliphatic hydrophobic. Loss of H-bonding.
Position in the protein
Connecting loop · position 461 (pLDDT 86).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| = 0.30. AlphaMissense 0.30 below threshold but Wolfram 1 confirms pathogenicity.

Mechanism: loss of T461-E462 H-bond. Therapeutic strategy: same E462 loop microregion as E462G.

Why this matters

T461I + E462G are sister variants at adjacent positions — both disrupt the T461-E462 H-bond geometry.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the T461I 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 T461I PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Natural variant461463 · in WFS1