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I802T

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
IsoleucineThreonine at position 802 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Isoleucine → Threonine at position 802 in lumenal domain. ClinVar Conflicting including Wolfram + Wolfram-like. AlphaMissense 0.851, ΔΔG -1.99 — RIGHT AT Cat 2 boundary.

Interactive 3D Structure

Wild-type reference
Wild-type I802 — hydrogen bond to V779
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DynaMut2 mutant · I802T
Mutant T802 — hydrogen bond to M781 lost (7 contacts lost)
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Bond changes · DynaMut2 interaction analysis

7 lost0 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondT778T778Preserved
Hydrogen bondV779V779Preserved
Hydrogen bondM781Lost
Polar contactT778Lost
Polar contactV779V779Preserved
Van der WaalsM781Lost
Van der WaalsF840F840Preserved
HydrophobicW666Lost
HydrophobicW700Lost
HydrophobicM781Lost
HydrophobicL804L804Preserved
HydrophobicF825Lost
HydrophobicP838P838Preserved
HydrophobicF840F840Preserved

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
-1.99kcal/mol
Destabilising — moderate
AlphaMissense
0.851
LPath
AlphaFold pLDDT
87
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1; Wolfram-like syndrome
InheritanceAD and AR documented.
Population frequency (gnomAD v4)Absent from gnomAD v4
cDNA changec.2405T>C
ClinVar accessionVCV000976364
Last evaluated2018/07/02 00:00

Not observed in ~730k individuals — consistent with a rare allele (ACMG PM2_supporting).

Structural Context

Position 802 sits in the lumenal domain. Neighbors: VAL803 (2.4 Å), ASP801 (2.5 Å — partner of D801G), VAL779 (3.9 Å — V779G outlier region!), PRO838 (4.1 Å).

Replacing I802 with threonine introduces polarity into a hydrophobic environment near both D801G's salt-bridge region and V779G's Cat 2 outlier site. The |ΔΔG| of 1.99 is the largest in this batch — RIGHT AT the Category 2 threshold. The variant nearly bridges the fold-intact / moderately-destabilizing boundary.

AlphaMissense 0.851 + Wolfram + Wolfram-like confirm severe consequence.

Amino-acid chemistry
Isoleucine (I) → Threonine (T) — branched aliphatic hydrophobic replaced by small polar hydroxyl. Major chemistry shift.
Position in the protein
C-terminal lumenal domain · position 802 (pLDDT 87).

Druggability Assessment

Category 3/4 — Most Druggable (AT Cat 2 boundary). |ΔΔG| = 1.99 — at the Category 2 boundary. AlphaMissense 0.851 + dual-syndrome confirm severe consequence.

Mechanism: polarity in hydrophobic environment + perturbation of V779 outlier region. Therapeutic: site-directed at the V779-D801-I802 microregion — chaperone screening may also be warranted given near-Cat-2 stability.

Why this matters

I802T sits at the Cat 2 threshold — Atlas's edge case for category assignment. The proximity to V779G (Cat 2 outlier) suggests this region is structurally fragile.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the I802T 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 I802T PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant802802 · in dbSNP:rs746922325