RareResearch.AI
← Back to atlas

E824K

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
GlutamateLysine at position 824 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Glutamate → Lysine at position 824 in lumenal domain. ClinVar Conflicting including DFNA6. AlphaMissense 0.566 (borderline), ΔΔG -0.13.

Interactive 3D Structure

Wild-type reference
Wild-type E824 — hydrogen bond to S846
Fullscreen ↗
DynaMut2 mutant · E824K
Mutant K824 — hydrophobic contact to T701 lost
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

0 lost0 gained9 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondW700W700Preserved
Hydrogen bondI845I845Preserved
Hydrogen bondS846S846Preserved
Polar contactW700W700Preserved
Polar contactI845I845Preserved
Polar contactS846S846Preserved
CarbonylI845I845Preserved
HydrophobicT701T701Preserved
HydrophobicL848L848Preserved

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
-0.13kcal/mol
Destabilising — mild
AlphaMissense
0.566
LPath
AlphaFold pLDDT
90
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsAutosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceDFNA6.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0047%
cDNA changec.2470G>A
ClinVar accessionVCV000166612
Last evaluated2024/03/21 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 824 in lumenal domain. Neighbors: PHE825 (2.4 Å — W700-F825 π-stacking partner!), ILE823 (2.5 Å), TRP700 (3.5 Å — direct contact with W700 Cat 2 region!), SER846 (3.7 Å).

E824 sits in direct contact with W700 and F825 — the same aromatic cluster identified in W700C and T699P Atlas cards. The wild-type E824 negative charge contributes to this microregion. Charge-flipping to K824 disrupts the local environment supporting W700-F825 π-stacking.

Mild ΔΔG; AM 0.566 borderline + DFNA6 confirm severe consequence.

Amino-acid chemistry
Glutamate (E) → Lysine (K) — charge reversal.
Position in the protein
C-terminal lumenal domain · position 824 (pLDDT 90).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.13. AlphaMissense 0.566 borderline + DFNA6 confirm severe consequence.

Mechanism: charge-flip in the W700-F825 aromatic cluster. Therapeutic: same W700-F825 microregion (with W700S/C, T699P/M).

Why this matters

E824K is the FIFTH variant converging on the W700-F825 π-stacking microregion. Drug discovery here has unusually dense convergence.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the E824K 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 E824K PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal