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R685H

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
ArginineHistidine at position 685 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Arginine → Histidine at position 685 — SAME position as R685P (Atlas card flagship pathogenic-stabilising variant) and R685C. ClinVar Conflicting with broad spectrum — Wolfram, Cataract 41, DFNA6. AlphaMissense 0.12 (below threshold) — AM under-call. DynaMut2 ΔΔG -1.18 (substantial).

Interactive 3D Structure

Wild-type reference
Wild-type R685 — hydrogen bond to L689
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DynaMut2 mutant · R685H
Mutant H685 — energy-minimized; local contact network preserved
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Bond changes · DynaMut2 interaction analysis

0 lost0 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondN682N682Preserved
Hydrogen bondI688I688Preserved
Hydrogen bondL689L689Preserved
Polar contactN682N682Preserved
Polar contactM683M683Preserved
Polar contactI688I688Preserved
Polar contactL689L689Preserved
Van der WaalsM683M683Preserved

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
-1.18kcal/mol
Destabilising — moderate
AlphaMissense
0.120
LBen
AlphaFold pLDDT
90
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1; Cataract 41; Autosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceBroad multi-phenotype.
Population frequency (gnomAD v4)Low frequency · AF 0.016%
cDNA changec.2054G>A
ClinVar accessionVCV000215395
Last evaluated2025/08/09 00:00

Observed in the general population.

Structural Context

Position 685 same neighbors as R685P/R685C: THR686 (2.5 Å), ALA684 (2.5 Å — A684T/V/G), ASN682 (3.5 Å), MET683 (4.3 Å), GLN687 (4.4 Å — Q687H).

R685H is the THIRD substitution at position 685 (with R685P, R685C). Partial charge reduction. |ΔΔG| 1.18 substantial; AM 0.12 under-call; multi-phenotype confirms.

Amino-acid chemistry
Arginine (R) → Histidine (H) — long positively-charged amine replaced by smaller titratable aromatic.
Position in the protein
C-terminal lumenal domain · position 685 (pLDDT 90). Same as R685P, R685C.

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 1.18 substantial. AlphaMissense 0.12 below threshold but THREE phenotypes + substantial ΔΔG confirm pathogenicity.

Mechanism: partial charge loss in the dense 684-688 cluster. Therapeutic: same cluster target as R685P, R685C, A684T/V/G, Q687H.

Why this matters

R685H is the THIRD substitution at position 685. The 684-688 cluster now contains 7+ variants — densest hub in the Atlas.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R685H 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 R685H PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant685685 · in DFNA6; dbSNP:rs142668478