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D171N

Category 4 — Stable Fold, Function DisruptedConflictingCytoplasmic · predictedEditorial
AspartateAsparagine at position 171 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Aspartate → Asparagine at position 171 in N-terminal cytoplasmic domain. ClinVar Conflicting. AlphaMissense 0.20 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.08 (neutral).

Interactive 3D Structure

Wild-type reference
Wild-type D171 — ionic bond to R174
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DynaMut2 mutant · D171N
Mutant N171 — ionic bond contact to R174 lost
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Bond changes · DynaMut2 interaction analysis

1 lost1 gained4 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondR174Lost
Hydrogen bondR174R174Preserved
Hydrogen bondA175A175Preserved
Polar contactR174R174Preserved
Polar contactA175A175Preserved
Van der WaalsA175Gained

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
-0.08kcal/mol
Destabilising — mild
AlphaMissense
0.199
LBen
AlphaFold pLDDT
85
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditions(no specific conditions catalogued)
InheritanceNot specified.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0043%
cDNA changec.511G>A
ClinVar accessionVCV001330801
Last evaluated2025/10/24 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 171 in cytoplasmic domain. Neighbors: THR170 (2.5 Å), LEU172 (2.5 Å), ARG174 (3.8 Å — R174 partner of R177C cluster region). The R174-D171 likely salt bridge.

D171N eliminates negative charge while preserving amide H-bond. R174 loses salt-bridge partner. ΔΔG ≈ 0; AM 0.20 under-call. ClinVar Conflicting with low evidence.

Amino-acid chemistry
Aspartate (D) → Asparagine (N) — carboxylate replaced by amide. Charge lost; H-bonding preserved.
Position in the protein
N-terminal cytoplasmic domain · position 171 (pLDDT 85).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call, low evidence). ΔΔG ≈ 0. AlphaMissense 0.20 below threshold. Limited clinical evidence.

Mechanism: loss of D171-R174 salt bridge. Therapeutic: same R174/R177 cluster microregion.

Why this matters

D171N targets the R174 microregion that R177C also disrupts.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the D171N 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 D171N PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A
Natural variant171171 · in DFNA6; dbSNP:rs758281375