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D866N

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
AspartateAsparagine at position 866 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Aspartate → Asparagine at position 866. ClinVar Conflicting including monogenic diabetes + Wolfram. AlphaMissense 0.18 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.59.

Interactive 3D Structure

Wild-type reference
Wild-type D866 — ionic bond to K862
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DynaMut2 mutant · D866N
Mutant N866 — ionic bond to K862 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost1 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondK862Lost
Hydrogen bondK862Lost
Hydrogen bondE864Lost
Hydrogen bondS869S869Preserved
Hydrogen bondT870T870Preserved
Polar contactK862K862Preserved
Polar contactE864E864Preserved
Polar contactR868Lost
Polar contactS869S869Preserved
Polar contactT870T870Preserved
Van der WaalsR868R868Preserved
Van der WaalsT870Gained

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
-0.59kcal/mol
Destabilising — mild
AlphaMissense
0.176
LBen
AlphaFold pLDDT
64
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Reduced confidence · Lumenal
  • pLDDT 63.5 below 70

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 conditionsMonogenic diabetes; Wolfram syndrome 1
InheritanceMonogenic diabetes + Wolfram.
Population frequency (gnomAD v4)Low frequency · AF 0.084%
cDNA changec.2596G>A
ClinVar accessionVCV000178655
Last evaluated2026/01/28 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Wolfram-like syndrome (autosomal dominant, OMIM 614296); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); Cataract 41 (autosomal dominant, OMIM 116400); WFS1-related spectrum (unresolved mode) (dominant or recessive). ClinVar records this under a WFS1-spectrum label that spans both disease families — recessive Wolfram syndrome (biallelic) and the dominant WFS1-related disorders. The label does not resolve which applies, and it is not a severity statement.

  • WFS1-related diabetesautosomal dominantsubmitted as: Monogenic diabetes; Type 2 diabetes mellitus
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1; Wolfram syndrome 1
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6; Autosomal dominant nonsyndromic hearing loss 6
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • 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 0.084% · 1,362 / 1,613,118 alleles
Homozygotes
13
Highest-frequency population
East Asian · AF 1.81%

Highest in East Asian: AF 1.81% (811 of 44,886 alleles), 21.4x the global figure. The global AF describes the general population, not the at-risk group.

13 homozygotes reported in gnomAD v4 (10 East Asian; 2 South Asian; 1 Remaining individuals). gnomAD excludes individuals with severe pediatric disease, so homozygotes in this dataset argue against a fully penetrant severe recessive phenotype and belong in the clinical picture. This is interpretive signal, not a classification.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
East Asian1.81%811 / 44,88610~1 in 28
African / African American0.274%206 / 75,0740~1 in 180
South Asian0.159%145 / 91,0742~1 in 310
Remaining individuals0.158%99 / 62,4981~1 in 320
Middle Eastern0.049%3 / 6,0620~1 in 1010
Admixed American0.027%16 / 60,0280~1 in 1880
European (non-Finnish)0.0069%82 / 1,179,9980~1 in 7200

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 866 in C-terminal cluster. Neighbors: TRP867 (2.5 Å), HIS865 (2.5 Å — partner of E864K via H865), ARG868 (4.3 Å — R868H!). The 864-868 cluster has E864K, H865 (E864K's neighbor), D866N (this card), R868H — four variants/positions in five residues.

D866N charge loss in dense multi-variant cluster. AM 0.18 under-call; multi-phenotype confirms.

Amino-acid chemistry
Aspartate (D) → Asparagine (N) — charge loss; H-bonding preserved.
Position in the protein
C-terminal lumenal domain · position 866 (pLDDT 64 borderline).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.59. AlphaMissense 0.18 below threshold but multi-phenotype confirms.

Mechanism: charge loss in dense 864-868 cluster. Therapeutic: same C-terminal cluster.

Why this matters

D866N extends the 864-868 multi-variant cluster — 5+ Atlas variants now in this 5-residue stretch.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the D866N 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 D866N PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal