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A150V

Category 4 — Stable Fold, Function DisruptedConflictingCytoplasmic · predictedEditorial
AlanineValine at position 150 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Alanine → Valine at position 150 in N-terminal cytoplasmic domain. ClinVar Conflicting including T2D. AlphaMissense 0.13 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.94 (substantial).

Interactive 3D Structure

Wild-type reference
Wild-type A150 — hydrogen bond to R146
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DynaMut2 mutant · A150V
Mutant V150 — hydrogen bond contact to R147 lost
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Bond changes · DynaMut2 interaction analysis

1 lost1 gained5 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondR146R146Preserved
Hydrogen bondR147R147Preserved
Hydrogen bondR152Gained
Polar contactR146R146Preserved
Polar contactR147R147Preserved
Polar contactR152R152Preserved
Van der WaalsR152Lost

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
-0.94kcal/mol
Destabilising — mild
AlphaMissense
0.131
LBen
AlphaFold pLDDT
91
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Standard confidence · Cytoplasmic

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 conditionsType 2 diabetes mellitus
InheritanceT2D.
Population frequency (gnomAD v4)Low frequency · AF 0.013%
cDNA changec.449C>T
ClinVar accessionVCV000178585
Last evaluated2025/11/23 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). These are dominant WFS1-related disorders and are clinically distinct from classic recessive Wolfram syndrome. This classification is not a statement about Wolfram syndrome severity.

  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • 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 0.013% · 208 / 1,577,956 alleles
Homozygotes
0
Highest-frequency population
East Asian · AF 0.268%

Highest in East Asian: AF 0.268% (115 of 42,898 alleles), 20.3x 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.
East Asian0.268%115 / 42,8980~1 in 190
South Asian0.053%46 / 85,9940~1 in 930
Remaining individuals0.034%21 / 61,2020~1 in 1460
African / African American0.0040%3 / 74,2640~1 in 12380
European (non-Finnish)0.0020%23 / 1,162,1880~1 in 25260

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 150 in N-term. Neighbors: ASP151 (2.4 Å), LEU149 (2.5 Å), ARG147 (3.8 Å — same R147 as R146C cluster region).

A150V volume mismatch near R146-R147 cluster. |ΔΔG| 0.94; AM 0.13 under-call; T2D confirms.

Amino-acid chemistry
Alanine (A) → Valine (V) — small methyl replaced by branched aliphatic.
Position in the protein
N-terminal cytoplasmic domain · position 150 (pLDDT 91).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.94. AlphaMissense 0.13 below threshold but T2D + substantial ΔΔG confirm pathogenicity.

Mechanism: volume mismatch near R146/R147 cluster. Therapeutic: same 146-150 cytoplasmic microregion.

Why this matters

A150V joins R146C and R138C in the 138-150 cytoplasmic cluster.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A150V 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 A150V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A