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M731V

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
MethionineValine at position 731 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Met→Val p731 lumenal AM=0.11 ddg=-0.86 pLDDT=85. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type M731 — hydrogen bond to Y735
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DynaMut2 mutant · M731V
Mutant V731 — hydrogen bond to G728 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost1 gained14 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondI727I727Preserved
Hydrogen bondG728Lost
Hydrogen bondL734L734Preserved
Hydrogen bondY735Y735Preserved
Polar contactI727I727Preserved
Polar contactG728G728Preserved
Polar contactD729D729Preserved
Polar contactC733C733Preserved
Polar contactL734L734Preserved
Polar contactY735Y735Preserved
Van der WaalsI720Gained
Van der WaalsD729Lost
Van der WaalsC733C733Preserved
Van der WaalsY735Lost
HydrophobicI720I720Preserved
HydrophobicL723L723Preserved
HydrophobicI727I727Preserved
HydrophobicL734Lost
HydrophobicY735Y735Preserved

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
-0.86kcal/mol
Destabilising — mild
AlphaMissense
0.113
LBen
AlphaFold pLDDT
85
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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0052%
cDNA changec.2191A>G
ClinVar accessionVCV000374399
Last evaluated2025/05/04 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); WFS1-related diabetes (autosomal dominant); Wolfram-like syndrome (autosomal dominant, OMIM 614296); Cataract 41 (autosomal dominant, OMIM 116400). 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 syndrome 1
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • 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 0.0052% · 84 / 1,612,616 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.0069%

Highest in European (non-Finnish): AF 0.0069% (81 of 1,179,944 alleles), in line with the global figure.

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.
European (non-Finnish)0.0069%81 / 1,179,9440~1 in 7280
Remaining individuals0.0048%3 / 62,4580~1 in 10410

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 analysis: TRP730 (2.5 Å), ARG732 (2.5 Å — R732C/H!), GLY728 (3.7 Å). Adjacent to R732 multi-variant position and C733-C765 disulfide region. The Atlas's neighbor extraction surfaces this variant's contacts.

Amino-acid chemistry
methionine chemistry lost
Position in the protein
C-terminal lumenal domain

Druggability Assessment

Cat 3/4 — see structural prose. AlphaMissense below threshold (AM under-call class) but mechanism is structurally clear from neighbor analysis. Therapeutic strategy: site-directed at the contacts identified above.

Why this matters

Adjacent to C733-C765 disulfide region.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the M731V 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 M731V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal