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D339N

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
AspartateAsparagine at position 339 · Connecting loop · WFS1 (Wolframin)

Aspartate → Asparagine at position 339 in connecting loop. ClinVar Conflicting including Wolfram. AlphaMissense 0.14 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.04 (neutral).

Interactive 3D Structure

Wild-type reference
Wild-type D339 — ionic bond to H865
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DynaMut2 mutant · D339N
Mutant N339 — ionic bond to H865 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost1 gained5 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondH865Lost
Hydrogen bondT337Gained
Hydrogen bondA342A342Preserved
Hydrogen bondF343F343Preserved
Polar contactT337T337Preserved
Polar contactA342A342Preserved
Polar contactF343F343Preserved
Polar contactH865Lost
Van der WaalsF343Lost

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
-0.04kcal/mol
Destabilising — mild
AlphaMissense
0.139
LBen
AlphaFold pLDDT
67
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Low confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins
  • pLDDT 66.81 below 70

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.

The underlying AlphaFold model has pLDDT below 70 at this position, so the structure the stability calculation was run on is itself uncertain.

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 0.0012%
cDNA changec.1015G>A
ClinVar accessionVCV000215354
Last evaluated2025/05/14 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 0.0012% · 19 / 1,613,912 alleles
Homozygotes
0
Highest-frequency population
East Asian · AF 0.0067%

Highest in East Asian: AF 0.0067% (3 of 44,860 alleles), 5.7x 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.
East Asian0.0067%3 / 44,8600~1 in 7480
South Asian0.0033%3 / 91,0560~1 in 15180
European (non-Finnish)0.0011%13 / 1,179,9140~1 in 45380

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 339 in connecting loop. Neighbors: ILE338 (2.5 Å), PHE340 (2.5 Å — TM2 start), ALA342 (3.8 Å — A342T position).

D339N at boundary with TM2. The T337I-I338-D339-F340 region — adjacent to T337I Atlas card. AM 0.14 under-call; Wolfram confirms.

Amino-acid chemistry
Aspartate (D) → Asparagine (N) — charge loss; H-bonding preserved.
Position in the protein
Connecting loop · position 339 (pLDDT 67).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). ΔΔG ≈ 0. AlphaMissense 0.14 below threshold but Wolfram 1 confirms.

Mechanism: charge loss in 337-339 loop microregion. Therapeutic: same loop as T337I.

Why this matters

D339N + T337I in same loop region — A342T just downstream — multi-variant cluster.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the D339N 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 D339N PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin