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A844V

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
AlanineValine at position 844 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Alanine → Valine at position 844 in lumenal C-terminal region. ClinVar Conflicting including spastic ataxia. AlphaMissense 0.782, ΔΔG +0.09 (near-neutral).

Interactive 3D Structure

Wild-type reference
Wild-type A844 — hydrogen bond to S826
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DynaMut2 mutant · A844V
Mutant V844 — hydrogen bond to F825 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost1 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondF825Lost
Hydrogen bondS826S826Preserved
Hydrogen bondV861V861Preserved
Polar contactF825F825Preserved
Polar contactS826S826Preserved
Polar contactL842L842Preserved
Polar contactV861V861Preserved
CarbonylF825Lost
Van der WaalsF825Lost
Van der WaalsS826S826Preserved
Van der WaalsK862Gained
HydrophobicK862K862Preserved

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
0.09kcal/mol
Stabilising — mild
AlphaMissense
0.782
LPath
AlphaFold pLDDT
88
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsSpastic ataxia; Inborn genetic diseases
InheritanceSpastic ataxia documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0058%
cDNA changec.2531C>T
ClinVar accessionVCV000666957
Last evaluated2026/01/17 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 844 sits in the lumenal C-terminus. Neighbors: ILE845 (2.4 Å), LYS843 (2.4 Å — partner of K843 cluster from L842F), VAL861 (3.2 Å — long-range; near K862N), SER826 (3.7 Å).

The wild-type alanine provides minimal volume. Replacing it with valine introduces branched aliphatic into a pocket sized for alanine. The K843-A844-V861 microregion is perturbed. AM 0.782 + spastic ataxia confirm severe consequence.

Amino-acid chemistry
Alanine (A) → Valine (V) — small replaced by branched aliphatic. Modest volume increase.
Position in the protein
C-terminal lumenal domain · position 844 (pLDDT 88).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG near-neutral. AlphaMissense 0.782 + spastic ataxia confirm severe consequence.

Mechanism: volume mismatch in the K843-V861 long-range microregion. Therapeutic: site-directed at the C-terminal cluster (with L842F, K862N targets).

Why this matters

A844V joins L842F and K862N as variants in the K843-V861 long-range cluster.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A844V 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 A844V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal