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I802M

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
IsoleucineMethionine at position 802 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Isoleucine → Methionine at position 802 in lumenal domain. ClinVar Conflicting for DFNA6. AlphaMissense 0.35 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.44 kcal/mol (destabilising). Same position as I802T (Cat 2 boundary).

Interactive 3D Structure

Wild-type reference
Wild-type I802 — hydrogen bond to V779
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DynaMut2 mutant · I802M
Mutant M802 — hydrogen bond to M781 lost (5 contacts lost)
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Bond changes · DynaMut2 interaction analysis

5 lost3 gained9 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondT778Lost
Hydrogen bondV779V779Preserved
Hydrogen bondM781Lost
Hydrogen bondF825Gained
Polar contactT778Lost
Polar contactV779V779Preserved
Van der WaalsM781Lost
Van der WaalsF825Gained
Van der WaalsF840Lost
HydrophobicW666W666Preserved
HydrophobicW700W700Preserved
HydrophobicV779Gained
HydrophobicM781M781Preserved
HydrophobicL804L804Preserved
HydrophobicF825F825Preserved
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
-0.44kcal/mol
Destabilising — mild
AlphaMissense
0.346
Amb
AlphaFold pLDDT
87
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
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 conditionsAutosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceDFNA6 hearing loss.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0013%
cDNA changec.2406C>G
ClinVar accessionVCV000229642
Last evaluated2025/12/18 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); Cataract 41 (autosomal dominant, OMIM 116400); Wolfram-like syndrome (autosomal dominant, OMIM 614296); WFS1-related diabetes (autosomal dominant); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300). 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.

  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6; Autosomal dominant nonsyndromic hearing loss 6
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • 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)
AF 0.0013% · 21 / 1,611,502 alleles
Homozygotes
0
Highest-frequency population
Admixed American · AF 0.030%

Highest in Admixed American: AF 0.030% (18 of 59,974 alleles), 23.0x the global figure. The global AF describes the general population, not the at-risk group.

No homozygotes reported in gnomAD v4. For a recessive allele this is expected and is not, on its own, evidence either way.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
Admixed American0.030%18 / 59,9740~1 in 1670
Middle Eastern · under-sampled0.016%1 / 6,0820
European (non-Finnish)0.00017%2 / 1,179,2880~1 in 294820

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 same neighbors as I802T: VAL803 (2.4 Å), ASP801 (2.5 Å — partner of D801G), VAL779 (3.9 Å — V779G Cat 2 outlier region!). I802M is the second pathogenic substitution at position 802.

Where I802T introduced polarity (Cat 2 boundary ΔΔG of -1.99), I802M is conservative — flexible sulfur-containing hydrophobic replaces branched aliphatic hydrophobic. The fold absorbs the substitution more easily (|ΔΔG| 0.44).

AlphaMissense's 0.35 is below threshold (AM under-call). DFNA6 clinical evidence does not resolve pathogenicity (ClinVar: conflicting submissions). Both I802T and I802M perturb the V779-D801-I802 microregion that connects the V779G Cat 2 outlier to the D801G salt-bridge position.

Amino-acid chemistry
Isoleucine (I) → Methionine (M) — branched aliphatic replaced by flexible sulfur-containing hydrophobic. Conservative chemistry shift.
Position in the protein
C-terminal lumenal domain · position 802 (pLDDT 87). Same position as I802T.

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| = 0.44. AlphaMissense 0.35 below threshold and DFNA6 does not resolve it (ClinVar: conflicting submissions).

Mechanism: conservative chemistry shift in the V779-D801-I802 microregion. Therapeutic strategy: same target as I802T, V779G, V779M, D801G, K800E.

Why this matters

I802M is the 5th variant in the V779-D801-I802 microregion. The cluster's structural importance is now extensively documented across multiple variants.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the I802M 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 I802M PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

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