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D367Y

Category 3/4 — Most DruggableConflictingTransmembrane · predictedEditorial
AspartateTyrosine at position 367 · Connecting loop · WFS1 (Wolframin)

Aspartate → Tyrosine at position 367 in a connecting loop. ClinVar Conflicting including Wolfram syndrome 1. AlphaMissense 0.685, ΔΔG +0.53 STABILISING.

Interactive 3D Structure

Wild-type reference
Wild-type D367 — ionic bond to H401
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DynaMut2 mutant · D367Y
Mutant Y367 — ionic bond to H401 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost11 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondH401Lost
Hydrogen bondK363K363Preserved
Hydrogen bondV364Lost
Hydrogen bondA370A370Preserved
Hydrogen bondW371W371Preserved
Hydrogen bondP404Lost
Polar contactK363K363Preserved
Polar contactV364V364Preserved
Polar contactA370A370Preserved
Polar contactW371W371Preserved
Polar contactH401Gained
Aromatic / πF264Gained
Aromatic / πH401Gained
CarbonylA370A370Preserved
Van der WaalsK363Lost
Van der WaalsK369Gained
Van der WaalsH401Gained
HydrophobicI259Gained
HydrophobicF264Gained
HydrophobicK363Gained
HydrophobicA370Gained
HydrophobicH401Gained
HydrophobicP404Gained

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
0.53kcal/mol
Stabilising — mild
AlphaMissense
0.685
LPath
AlphaFold pLDDT
80
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable
ΔΔG confidence: Reduced confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins

This variant sits in the transmembrane band. The stability value was computed with a predictor benchmarked on water-soluble proteins. Published benchmarks of ddG predictors on membrane proteins report correlation below 0.4. Treat the magnitude as unreliable and the sign as weak evidence only.

Do not rank this value against a variant from a different region on ΔΔG alone.

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0020%
cDNA changec.1099G>T
ClinVar accessionVCV001810357
Last evaluated2023/02/13 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram syndrome (classic) (autosomal recessive, OMIM 222300). Classic Wolfram syndrome is recessive: a single copy does not produce it, and this classification says nothing about a carrier's own risk.

  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Submitters conflict on this classification (1★). Treat the conditions above as submitted, not as documented phenotypes.
  • 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 0.0020% · 3 / 152,078 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.0044%

Highest in European (non-Finnish): AF 0.0044% (3 of 68,028 alleles), 2.2x 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.
European (non-Finnish)0.0044%3 / 68,0280~1 in 11340

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 367 in connecting loop. Neighbors: GLN366 (2.5 Å), SER368 (2.5 Å), VAL364 (3.7 Å — near K363T), LYS363 (3.8 Å — partner of K363T!). The K363 contact is structurally significant: D367 wild-type likely salt-bridges with K363.

Replacing D367 with tyrosine eliminates the salt-bridge potential. The variant fold stabilises (+0.53) because the aromatic ring packs into the local environment. AM 0.685 + Wolfram 1 confirm severe consequence. Mechanism is loss of D367-K363 salt bridge.

Amino-acid chemistry
Aspartate (D) → Tyrosine (Y) — small negatively-charged carboxylate replaced by large aromatic phenol. Charge loss + aromatic introduction.
Position in the protein
Connecting loop · position 367 (pLDDT 80).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG = +0.53 stabilising. AlphaMissense 0.685 + Wolfram 1 confirm severe consequence.

Mechanism: loss of D367-K363 salt bridge. Therapeutic: same K363 microregion as K363T.

Why this matters

D367Y + K363T are sister variants at the K363-D367 salt-bridge pair.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the D367Y 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 D367Y PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin