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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
ΔΔG confidence: Standard confidence · Lumenal

This variant is in a water-facing region, which is the regime the stability predictor was built and benchmarked for. Ordinary predictor error still applies.

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).

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Wolfram-like syndrome (autosomal dominant, OMIM 614296). These are two clinically distinct disease families: classic Wolfram syndrome requires two affected copies, while the dominant WFS1-related disorders do not. One label covers both, and it does not tell you which applies to a given person.

  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • Submitters conflict on this classification (1★). Treat the conditions above as submitted, not as documented phenotypes.
  • Phenotype scope is interpretation of submitted records. The computed values on this card (AlphaMissense, DynaMut2 ΔΔG, pLDDT) are measurements of the model, not of a patient.
  • Presentation cannot be generalised from neighbouring variants: one nucleotide over can present very differently. Structural similarity supports a mechanistic hypothesis, never a phenotype claim.

Review status: 1★ conflicting classifications. ClinVar "conditions" is the union of all submissions; 0–1★ entries are submitted conditions, not documented phenotypes.

Population frequency
Global (all ancestries)
Absent from gnomAD v4
Homozygotes
Not available

No population breakdown — the variant is absent from gnomAD v4, so no ancestry group has an observed frequency.

No homozygotes — the variant is absent from gnomAD v4 altogether.

Source: gnomAD r4, retrieved 2026-08-21. Values are allele frequencies. The carrier estimate is derived from the allele frequency (Hardy-Weinberg, ~2·AF) and is an approximation, not a published figure — where a published carrier frequency exists it outranks this column and is cited on the card.

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