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K800E

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
LysineGlutamate at position 800 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Lysine → Glutamate at position 800 in lumenal domain. ClinVar Conflicting. AlphaMissense 0.778, ΔΔG -0.40. pLDDT 72 borderline. Same K800-D801 salt-bridge pair as D801G.

Interactive 3D Structure

Wild-type reference
Wild-type K800 — ionic bond to E794
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DynaMut2 mutant · K800E
Mutant E800 — ionic bond to E794 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost1 gained3 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondE794Lost
Hydrogen bondE794Lost
Hydrogen bondD797D797Preserved
Polar contactW666Gained
Polar contactE794Lost
Polar contactD797D797Preserved
Polar contactV798V798Preserved
HydrophobicD797Lost

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
-0.40kcal/mol
Destabilising — mild
AlphaMissense
0.778
LPath
AlphaFold pLDDT
72
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable
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 conditions(no specific conditions catalogued)
InheritanceNot specified.
Population frequency (gnomAD v4)Low frequency · AF 0.022%
cDNA changec.2398A>G
ClinVar accessionVCV000215400
Last evaluated2025/11/03 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity, but does not state what it is classified for. ClinVar records the condition only as "not specified", "not provided". A classification without a phenotype and an inheritance mode cannot be read as a statement about Wolfram syndrome risk or severity.

  • 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 0.022% · 352 / 1,612,232 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.028%

Highest in European (non-Finnish): AF 0.028% (333 of 1,179,658 alleles), in line with the global figure.

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)0.028%333 / 1,179,6580~1 in 1770
Remaining individuals0.014%9 / 62,4820~1 in 3470
Admixed American0.012%7 / 60,0040~1 in 4290
African / African American0.0040%3 / 75,0700~1 in 12510

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 800 sits adjacent to D801. Neighbors: ASP801 (2.5 Å — D801G Atlas card!), THR799 (2.5 Å), ASP797 (3.7 Å), VAL798 (4.3 Å).

The wild-type K800-D801 salt bridge (referenced in D801G Atlas card) breaks when K800 becomes E800: now TWO adjacent glutamates (E800, D801) with no positive charge to stabilize. The local electrostatic environment is transformed. ΔΔG 0.40 + AM 0.778 confirm severe consequence.

Amino-acid chemistry
Lysine (K) → Glutamate (E) — positively-charged amine replaced by negatively-charged carboxylate. Charge reversal.
Position in the protein
C-terminal lumenal domain · position 800 (pLDDT 72).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.40. AlphaMissense 0.778 confirms severe consequence.

Mechanism: K800-D801 salt bridge broken by charge-flip at K800. Therapeutic: same K800-D801 microregion as D801G.

Why this matters

K800E + D801G are sister variants at the salt-bridge pair. Two convergent variant targets at the same ionic contact.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the K800E 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 K800E PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal