RareResearch.AI
← Back to atlas

G76V

Category 5 — IDR ExclusionConflictingCytoplasmic · predictedEditorial
GlycineValine at position 76 · N-terminal intrinsically disordered region (1-86) · WFS1 (Wolframin)

Gly→Val p76 IDR AM=0.08 ddg=-1.11 pLDDT=26. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type G76 — hydrogen bond to A74
Fullscreen ↗
DynaMut2 mutant · G76V
Mutant V76 — hydrogen bond contact to A74 lost
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

1 lost1 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondA74A74Preserved
Polar contactA74A74Preserved
Van der WaalsA74Lost
HydrophobicA74Gained

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
-1.11kcal/mol
Destabilising — moderate
AlphaMissense
0.084
LBen
AlphaFold pLDDT
26
model confidence
Schema
Cat 5
Category 5 — IDR Exclusion
ΔΔG confidence: Reduced confidence · Cytoplasmic
  • pLDDT 26.47 below 70

The underlying AlphaFold model has pLDDT below 70 at this position, so the structure the stability calculation was run on is itself uncertain.

Do not rank this value against a variant from a different region on ΔΔG alone.

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)Low frequency · AF 0.035%
cDNA changec.227G>T
ClinVar accessionVCV000215375
Last evaluated2026/01/05 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant); WFS1-related spectrum (unresolved mode) (dominant or recessive); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965). 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 diabetesautosomal dominantsubmitted as: Monogenic diabetes
  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • 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 0.035% · 541 / 1,558,848 alleles
Homozygotes
0
Highest-frequency population
African / African American · AF 0.656%

Highest in African / African American: AF 0.656% (483 of 73,644 alleles), 18.9x 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.
African / African American0.656%483 / 73,6440~1 in 76
Remaining individuals0.058%35 / 60,3880~1 in 860
Admixed American0.034%18 / 52,1880~1 in 1450
European (non-Finnish)0.00043%5 / 1,152,2240~1 in 115220

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: THR77 (2.4 Å), ASP75 (2.5 Å), ALA74 (4.3 Å). pLDDT 26 deep IDR. Substantial ΔΔG but untrustworthy in this region. The Atlas's neighbor extraction surfaces this variant's contacts and connects them to the broader multi-variant target landscape.

Amino-acid chemistry
glycine flexibility lost
Position in the protein
N-terminal IDR

Druggability Assessment

Cat 5 IDR — see structural prose. AlphaMissense below threshold (AM under-call class) but mechanism is structurally identified. Therapeutic strategy: site-directed at contacts identified above, or wet-lab validation if pLDDT borderline/below 50.

Why this matters

Deep IDR glycine removal.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the G76V 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 G76V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A
Region186 · Disordered