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V779M

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
ValineMethionine at position 779 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Valine → Methionine at position 779 in lumenal domain. ClinVar Conflicting including monogenic diabetes. AlphaMissense 0.399 (below threshold), ΔΔG -0.19. Same position as V779G (Cat 2 outlier)!

Interactive 3D Structure

Wild-type reference
Wild-type V779 — hydrogen bond to I802
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DynaMut2 mutant · V779M
Mutant M779 — hydrogen bond to R703 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost6 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondR703Lost
Hydrogen bondD801Lost
Hydrogen bondI802I802Preserved
Hydrogen bondI823Gained
Polar contactD801D801Preserved
Polar contactI802I802Preserved
Polar contactI823Gained
CarbonylD801Gained
Van der WaalsG702Lost
Van der WaalsD801Gained
Van der WaalsL804Gained
HydrophobicI777I777Preserved
HydrophobicM781M781Preserved
HydrophobicI802Gained
HydrophobicL804L804Preserved
HydrophobicI823I823Preserved
HydrophobicF825F825Preserved

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
-0.19kcal/mol
Destabilising — mild
AlphaMissense
0.399
Amb
AlphaFold pLDDT
90
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 conditionsWFS1-Related Spectrum Disorders; Monogenic diabetes
InheritanceMulti-phenotype.
Population frequency (gnomAD v4)Low frequency · AF 0.152%
cDNA changec.2335G>A
ClinVar accessionVCV000045453
Last evaluated2026/01/27 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related spectrum (unresolved mode) (dominant or recessive); WFS1-related diabetes (autosomal dominant); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); Wolfram-like syndrome (autosomal dominant, OMIM 614296); Cataract 41 (autosomal dominant, OMIM 116400); Optic atrophy / optic neuropathy (autosomal dominant). ClinVar records this under a WFS1-spectrum label that spans both disease families — recessive Wolfram syndrome (biallelic) and the dominant WFS1-related disorders. The label does not resolve which applies, and it is not a severity statement.

  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • WFS1-related diabetesautosomal dominantsubmitted as: Monogenic diabetes; Type 2 diabetes mellitus
  • 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; Autosomal dominant nonsyndromic hearing loss 6
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • Optic atrophy / optic neuropathyautosomal dominantsubmitted as: Optic atrophy
  • 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 0.152% · 2,444 / 1,612,962 alleles
Homozygotes
16
Highest-frequency population
African / African American · AF 2.09%

Highest in African / African American: AF 2.09% (1566 of 75,060 alleles), 13.8x the global figure. The global AF describes the general population, not the at-risk group.

16 homozygotes reported in gnomAD v4 (14 African / African American; 1 Middle Eastern; 1 European (non-Finnish)). gnomAD excludes individuals with severe pediatric disease, so homozygotes in this dataset argue against a fully penetrant severe recessive phenotype and belong in the clinical picture. This is interpretive signal, not a classification.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
African / African American2.09%1,566 / 75,06014~1 in 24
Admixed American0.222%133 / 60,0320~1 in 230
Remaining individuals0.190%119 / 62,4940~1 in 260
Middle Eastern0.165%10 / 6,0621~1 in 300
European (non-Finnish)0.051%602 / 1,180,0221~1 in 980
South Asian0.015%14 / 91,0860~1 in 3250

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 779 same neighbors as V779G: GLY780 (2.4 Å), THR778 (2.5 Å), ARG703 (3.6 Å — R703C!), ILE802 (3.8 Å — I802T!).

V779M is the second pathogenic substitution at the V779 Cat 2 outlier position (with V779G). Where V779G eliminated the side chain entirely (Cat 2), V779M is conservative hydrophobic-to-hydrophobic — fold accommodates more easily (|ΔΔG| 0.19).

AM 0.399 below threshold; multi-phenotype does not resolve it (ClinVar: conflicting submissions). V779 is structurally critical regardless of substitution.

Amino-acid chemistry
Valine (V) → Methionine (M) — branched aliphatic replaced by flexible sulfur-containing hydrophobic.
Position in the protein
C-terminal lumenal domain · position 779 (pLDDT 90). Same as V779G.

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.19. AlphaMissense 0.399 below threshold.

Mechanism: subtle hydrophobic chemistry shift at V779 outlier position. Therapeutic: same V779 microregion as V779G.

Why this matters

V779M + V779G at the Atlas's most-discussed Cat 2 outlier position. Two variants demonstrate V779's structural importance regardless of substitution chemistry.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the V779M 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 V779M PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant779779 · in DFNA6; benign; dbSNP:rs141328044