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K862N

Category 3/4 — Most DruggableLikely pathogenicLumenal · predictedσ-1 candidateEditorial
LysineAsparagine at position 862 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Lysine → Asparagine at position 862 in wolframin's C-terminal lumenal domain. ClinVar Likely pathogenic for Wolfram syndrome 1. AlphaMissense 0.981, DynaMut2 ΔΔG -0.20 kcal/mol (mild destabilising). pLDDT 64 borderline. A charge-loss variant near the C-terminus.

Interactive 3D Structure

Wild-type reference
Wild-type K862 — ionic bond to D866
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DynaMut2 mutant · K862N
Mutant N862 — ionic bond to D866 lost (8 contacts lost)
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Bond changes · DynaMut2 interaction analysis

8 lost0 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondE864Lost
Ionic bondD866Lost
Hydrogen bondK843K843Preserved
Hydrogen bondE864Lost
Hydrogen bondD866Lost
Polar contactK843Lost
Polar contactE864Lost
Polar contactD866Lost
HydrophobicA844A844Preserved
HydrophobicE864Lost

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
-0.20kcal/mol
Destabilising — mild
AlphaMissense
0.981
LPath
AlphaFold pLDDT
64
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationLikely pathogenic
Review statuscriteria provided, single submitter
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1 (AR) documented.
Population frequency (gnomAD v4)Absent from gnomAD v4
cDNA changec.2586G>C
ClinVar accessionVCV003338038
Last evaluated2024/08/20 00:00

Not observed in ~730k individuals — consistent with a rare allele (ACMG PM2_supporting).

Structural Context

Position 862 sits near the C-terminus of wolframin's lumenal domain. The AlphaFold model places K862 within 5 Å of ILE863 (2.5 Å), VAL861 (2.5 Å), LYS843 (3.5 Å — second nearby lysine), ALA844 (4.0 Å), and GLU864 (4.6 Å — partner of E864K Atlas card).

The wild-type lysine likely forms a long-range salt bridge with E864 or contributes positive charge to a C-terminal surface patch including K843. Replacing K862 with N862 eliminates the positive charge and shortens the side chain. The mild |ΔΔG| of 0.20 reflects fold accommodation; AlphaMissense's 0.981 + Wolfram syndrome 1 clinical evidence confirm severe functional consequence.

Notably E864K (Atlas card adjacent) and K862N together establish the K862-E864 microregion as having multiple pathogenic variants — drug discovery here has convergent rescue opportunities.

Amino-acid chemistry
Lysine (K) → Asparagine (N) — large positively-charged primary amine replaced by neutral polar amide. Loss of charge and long side chain.
Position in the protein
C-terminal lumenal domain · position 862 near the C-terminus (pLDDT 64).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.20 — fold survives. AlphaMissense 0.981 + Wolfram 1 confirm severe functional consequence.

The mechanism is C-terminal surface charge loss. Therapeutic strategy: site-directed at the K862-E864 microregion — same target region as E864K.

Why this matters

K862N + E864K together establish the C-terminal lumenal region as a multi-variant drug target. Two charge-flip-like variants in adjacent positions.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the K862N 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 K862N PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal