RareResearch.AI
← Back to atlas

R653C

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

Arginine → Cysteine at position 653 in lumenal domain. ClinVar Conflicting. AlphaMissense 0.476 (below threshold), ΔΔG +0.22. pLDDT 61 borderline.

Interactive 3D Structure

Wild-type reference
Wild-type R653 — hydrogen bond to Y650
Fullscreen ↗
DynaMut2 mutant · R653C
Mutant C653 — hydrogen bond contact to F649 lost
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

1 lost1 gained9 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondF649F649Preserved
Hydrogen bondY650Y650Preserved
Hydrogen bondG656G656Preserved
Polar contactF649F649Preserved
Polar contactY650Y650Preserved
Polar contactV651V651Preserved
Polar contactG656G656Preserved
Van der WaalsY650Gained
Van der WaalsV651V651Preserved
Van der WaalsE655E655Preserved
HydrophobicF649Lost

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
0.22kcal/mol
Stabilising — mild
AlphaMissense
0.476
Amb
AlphaFold pLDDT
61
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWFS1-Related Spectrum Disorders
InheritanceWFS1 spectrum.
Population frequency (gnomAD v4)Low frequency · AF 0.028%
cDNA changec.1957C>T
ClinVar accessionVCV000178597
Last evaluated2026/01/24 00:00

Observed in the general population.

Structural Context

Position 653 at the lumenal domain start. Neighbors: SER654 (2.5 Å), TYR652 (2.5 Å), TYR650 (3.6 Å — Y650H/Y650D/Y650C region!), PHE649 (3.8 Å — Y650 cluster).

R653C sits adjacent to the Y650 aromatic cluster (multiple Atlas variants at Y650). The introduced thiol could engage in aberrant disulfide chemistry. AM 0.476 below threshold but multi-phenotype + ClinVar Pathogenic raise concern.

Amino-acid chemistry
Arginine (R) → Cysteine (C) — charge loss + thiol introduction.
Position in the protein
C-terminal lumenal domain · position 653 at the start of the lumenal domain (pLDDT 61 borderline).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM below threshold, pLDDT borderline). ΔΔG +0.22. AlphaMissense 0.476 below threshold.

Mechanism: charge loss + thiol near Y650 cluster. Therapeutic: same Y650 microregion. Wet-lab validation recommended.

Why this matters

R653C joins the AM-under-call class. Adjacency to Y650 cluster makes it of interest despite the borderline signals.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R653C 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 R653C PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant653653 · in DFNA6; uncertain significance; also found in a patient with type 2 diabetes; uncertain significance; dbSNP:rs201064551