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R232H

Category 4 — Stable Fold, Function DisruptedConflictingCytoplasmic · predictedEditorial
ArginineHistidine at position 232 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Arginine → Histidine at position 232 in N-terminal cytoplasmic domain. ClinVar Conflicting including WFS1 spectrum + Wolfram. AlphaMissense 0.17 (below threshold) — AM under-call. DynaMut2 ΔΔG -1.27 (substantial destabilising).

Interactive 3D Structure

Wild-type reference
Wild-type R232 — hydrogen bond to R228
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DynaMut2 mutant · R232H
Mutant H232 — hydrogen bond to N373 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost3 gained11 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondR228R228Preserved
Hydrogen bondM229M229Preserved
Hydrogen bondS235S235Preserved
Hydrogen bondS236S236Preserved
Hydrogen bondN373N373Preserved
Polar contactR228R228Preserved
Polar contactM229M229Preserved
Polar contactL230L230Preserved
Polar contactS235S235Preserved
Polar contactS236S236Preserved
Polar contactY254Gained
Polar contactL263Lost
Polar contactN373Lost
Van der WaalsR228Lost
Van der WaalsL230Lost
Van der WaalsV234Gained
Van der WaalsY254Gained
HydrophobicL263L263Preserved

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
-1.27kcal/mol
Destabilising — moderate
AlphaMissense
0.172
LBen
AlphaFold pLDDT
76
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; Wolfram syndrome 1
InheritanceWFS1 spectrum.
Population frequency (gnomAD v4)Low frequency · AF 0.015%
cDNA changec.695G>A
ClinVar accessionVCV000907358
Last evaluated2026/01/01 00:00

Observed in the general population.

Structural Context

Position 232 in cytoplasmic domain. Neighbors: LEU233 (2.5 Å), GLU231 (2.5 Å — same E231 as R228H!), SER235 (3.5 Å). The R232-E231 salt bridge (with adjacent R228H Atlas card overlapping) creates a multi-variant cluster.

R232H + R228H both perturb the R227-R228-E231-R232 charged cluster. |ΔΔG| 1.27 substantial; AM 0.17 under-call; multi-phenotype confirms.

Amino-acid chemistry
Arginine (R) → Histidine (H) — charge partial-reduction.
Position in the protein
N-terminal cytoplasmic domain · position 232 (pLDDT 76).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 1.27. AlphaMissense 0.17 below threshold but multi-phenotype + substantial ΔΔG confirm pathogenicity.

Mechanism: charge partial-loss in R227-R228-E231-R232 cluster. Therapeutic: same cytoplasmic charged cluster as R228H.

Why this matters

R232H + R228H + R227 + E231 — four-position charged cluster, multi-variant target.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R232H 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 R232H PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A