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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
ΔΔG confidence: Standard confidence · Cytoplasmic

This variant is in a water-facing region, which is the regime the stability predictor was built and benchmarked for. Ordinary predictor error still applies.

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.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity, but does not state what it is classified for. ClinVar records the condition only as "not provided". A classification without a phenotype and an inheritance mode cannot be read as a statement about Wolfram syndrome risk or severity.

  • Phenotype scope is interpretation of submitted records. The computed values on this card (AlphaMissense, DynaMut2 ΔΔG, pLDDT) are measurements of the model, not of a patient.
  • Presentation cannot be generalised from neighbouring variants: one nucleotide over can present very differently. Structural similarity supports a mechanistic hypothesis, never a phenotype claim.

Review status: 1★ conflicting classifications. ClinVar "conditions" is the union of all submissions; 0–1★ entries are submitted conditions, not documented phenotypes.

Population frequency
Global (all ancestries)
AF 0.0043% · 70 / 1,613,210 alleles
Homozygotes
0
Highest-frequency population
Finnish · AF 0.040%

Highest in Finnish: AF 0.040% (25 of 63,184 alleles), 9.1x the global figure. The global AF describes the general population, not the at-risk group.

No homozygotes reported in gnomAD v4. For a recessive allele this is expected and is not, on its own, evidence either way.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
Finnish0.040%25 / 63,1840~1 in 1260
Admixed American0.032%19 / 60,0180~1 in 1580
South Asian0.0044%4 / 91,0400~1 in 11380
Remaining individuals0.0032%2 / 62,5040~1 in 15630
European (non-Finnish)0.0017%20 / 1,180,0140~1 in 29500

Source: gnomAD r4, retrieved 2026-08-21. Values are allele frequencies. The carrier estimate is derived from the allele frequency (Hardy-Weinberg, ~2·AF) and is an approximation, not a published figure — where a published carrier frequency exists it outranks this column and is cited on the card.

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