RareResearch.AI
← Back to atlas

E737K

Category 4 — Stable Fold, Function DisruptedConflictingLumenal · predictedσ-1 candidateEditorial
GlutamateLysine at position 737 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Glutamate → Lysine at position 737 in lumenal domain. ClinVar Conflicting including WFS1 spectrum. AlphaMissense 0.18 (below threshold) — AM under-call. DynaMut2 ΔΔG +0.10 (neutral). Same E737 contacted by G736R, G736S, C765R.

Interactive 3D Structure

Wild-type reference
Wild-type E737 — ionic bond to H766
Fullscreen ↗
DynaMut2 mutant · E737K
Mutant K737 — ionic bond to H766 lost (5 contacts lost)
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

5 lost0 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondH766Lost
Hydrogen bondH766H766Preserved
Hydrogen bondK769Lost
Polar contactC765Lost
Polar contactH766H766Preserved
CarbonylC765Lost
Van der WaalsC765Lost

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
0.10kcal/mol
Stabilising — mild
AlphaMissense
0.176
LBen
AlphaFold pLDDT
88
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Standard confidence · Lumenal

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 conditionsWFS1-Related Spectrum Disorders
InheritanceWFS1 spectrum.
Population frequency (gnomAD v4)Low frequency · AF 0.093%
cDNA changec.2209G>A
ClinVar accessionVCV000143132
Last evaluated2026/01/27 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related spectrum (unresolved mode) (dominant or recessive); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965). 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 spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • 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 0.093% · 1,504 / 1,612,666 alleles
Homozygotes
26
Highest-frequency population
East Asian · AF 2.89%

Highest in East Asian: AF 2.89% (1296 of 44,860 alleles), 31.0x the global figure. The global AF describes the general population, not the at-risk group.

26 homozygotes reported in gnomAD v4 (26 East Asian). 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 Asian2.89%1,296 / 44,86026~1 in 17
Ashkenazi Jewish0.182%54 / 29,5900~1 in 270
Remaining individuals0.061%38 / 62,4800~1 in 820
Middle Eastern0.033%2 / 6,0620~1 in 1520
Admixed American0.013%8 / 59,9900~1 in 3750
European (non-Finnish)0.0083%98 / 1,179,8960~1 in 6020
South Asian0.0077%7 / 91,0660~1 in 6500
African / African American · under-sampled0.0013%1 / 75,0440

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 737 — the E737 hub residue itself. Neighbors: ALA738 (2.4 Å), GLY736 (2.5 Å — G736R/G736S!), HIS766 (3.8 Å — same H766 as C765R neighbor).

E737K is the variant AT the hub position that G736R, G736S, and C765R all contact. Charge-flip disrupts the entire microregion's electrostatic geometry. Three convergent Atlas variants point at E737 from their neighbor analyses; now we have the variant at the hub itself.

AM 0.18 under-call; WFS1 spectrum confirms.

Amino-acid chemistry
Glutamate (E) → Lysine (K) — charge reversal.
Position in the protein
C-terminal lumenal domain · position 737 (pLDDT 88).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call, HUB residue). ΔΔG ≈ 0. AlphaMissense 0.18 below threshold and multi-phenotype does not resolve it (ClinVar: conflicting submissions).

Mechanism: charge-flip at E737 hub. Therapeutic: this is the second hub residue in the Atlas (with E431). Drug discovery targets E737 microregion.

Why this matters

E737K identifies E737 as a second multi-variant hub residue (G736R, G736S, C765R, E737K all converge here).
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the E737K 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 E737K PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant737737 · in dbSNP:rs147834269