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D267N

Category 5 — IDR ExclusionConflictingCytoplasmic · predictedEditorial
AspartateAsparagine at position 267 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Asp→Asn p267 N-term AM=0.11 ddg=+0.77 pLDDT=31. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type D267 — hydrogen bond to L265
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DynaMut2 mutant · D267N
Mutant N267 — van der waals contact to L265 lost
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Bond changes · DynaMut2 interaction analysis

1 lost0 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondL265L265Preserved
Polar contactL265L265Preserved
Van der WaalsL265Lost

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
0.77kcal/mol
Stabilising — mild
AlphaMissense
0.109
LBen
AlphaFold pLDDT
31
model confidence
Schema
Cat 5
Category 5 — IDR Exclusion
ΔΔG confidence: Reduced confidence · Cytoplasmic
  • pLDDT 31.11 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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Low frequency · AF 0.041%
cDNA changec.799G>A
ClinVar accessionVCV000166577
Last evaluated2026/01/21 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Wolfram-like syndrome (autosomal dominant, OMIM 614296); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); WFS1-related diabetes (autosomal dominant); Cataract 41 (autosomal dominant, OMIM 116400). These are two clinically distinct disease families: classic Wolfram syndrome requires two affected copies, while the dominant WFS1-related disorders do not. One label covers both, and it does not tell you which applies to a given person.

  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: 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
  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41

Not attributable to this variant: Lymphedema — 4p16.3 contiguous-gene/CNV submissions.

  • Submitters conflict on this classification (1★). Treat the conditions above as submitted, not as documented phenotypes.
  • Also submitted under Lymphedema — 4p16.3 contiguous-gene/CNV submissions, not attributable to this coding change.
  • 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 0.041% · 665 / 1,613,146 alleles
Homozygotes
0
Highest-frequency population
Amish · AF 0.219%

Highest in Amish: AF 0.219% (2 of 912 alleles), 5.3x 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.
Amish0.219%2 / 9120~1 in 230
European (non-Finnish)0.053%628 / 1,180,0320~1 in 940
Remaining individuals0.029%18 / 62,4720~1 in 1740
African / African American0.0093%7 / 74,9280~1 in 5350
Admixed American0.0083%5 / 60,0160~1 in 6000
East Asian0.0067%3 / 44,8920~1 in 7480
Finnish0.0032%2 / 63,2180~1 in 15800

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 analysis: ASP268 (2.5 Å — adjacent existing D), GLN266 (2.5 Å), LEU265 (4.2 Å). pLDDT 31 deep IDR. DynaMut2 untrustworthy. The Atlas's neighbor extraction surfaces this variant's contacts.

Amino-acid chemistry
charge loss, H-bond preserved
Position in the protein
N-terminal cytoplasmic IDR-boundary

Druggability Assessment

Category 5 — see structural prose. AlphaMissense below threshold (AM under-call class) but mechanism is structurally clear from neighbor analysis. Therapeutic strategy: site-directed at the contacts identified above.

Why this matters

deep IDR — wet-lab required.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the D267N 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 D267N PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A