RareResearch.AI
← Back to atlas

R457S

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
ArginineSerine at position 457 · Connecting loop · WFS1 (Wolframin)

Arginine → Serine at position 457 in connecting loop. ClinVar Conflicting including monogenic diabetes + spastic. AlphaMissense 0.515 (borderline), ΔΔG -0.82.

Interactive 3D Structure

Wild-type reference
Wild-type R457 — hydrogen bond to P453
Fullscreen ↗
DynaMut2 mutant · R457S
Mutant S457 — hydrogen bond contact to Y454 lost
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

1 lost2 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondP453P453Preserved
Hydrogen bondY454Y454Preserved
Hydrogen bondA460A460Preserved
Hydrogen bondT461T461Preserved
Polar contactP453P453Preserved
Polar contactY454Y454Preserved
Polar contactT455Gained
Polar contactA460A460Preserved
Polar contactT461T461Preserved
Polar contactL514Gained
HydrophobicL514Lost

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
-0.82kcal/mol
Destabilising — mild
AlphaMissense
0.515
Amb
AlphaFold pLDDT
88
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 disorder; Monogenic diabetes; Spastic ataxia
InheritanceMonogenic diabetes + WFS1 spectrum.
Population frequency (gnomAD v4)Low frequency · AF 0.047%
cDNA changec.1371G>T
ClinVar accessionVCV000215389
Last evaluated2025/11/18 00:00

Observed in the general population.

Structural Context

Position 457 in connecting loop. Neighbors: ALA458 (2.5 Å), ARG456 (2.5 Å — adjacent existing arginine!), TYR454 (3.7 Å), PRO453 (4.1 Å).

Replacing R457 with serine eliminates one of two adjacent arginines (R456 + R457). The local positively-charged surface character is reduced. ΔΔG 0.82 + AM 0.515 borderline + monogenic diabetes confirm severe consequence.

Amino-acid chemistry
Arginine (R) → Serine (S) — long positively-charged amine replaced by small polar hydroxyl. Loss of charge + side-chain length.
Position in the protein
Connecting loop · position 457 (pLDDT 88).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.82. AlphaMissense 0.515 borderline + multi-phenotype confirm severe consequence.

Mechanism: loss of R457 charge from R456-R457 cluster. Therapeutic: site-directed at the loop's charged surface.

Why this matters

R457S continues the charge-loss class in connecting loops.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R457S 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 R457S PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Natural variant457457 · in WFS1; dbSNP:rs113446173