RareResearch.AI
← Back to atlas

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
Fullscreen ↗
DynaMut2 mutant · A844V
Mutant V844 — hydrogen bond to F825 lost (3 contacts lost)
Fullscreen ↗

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
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 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.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Ataxia / spastic ataxia (inheritance not specified).

  • Ataxia / spastic ataxiainheritance not specifiedsubmitted as: Spastic ataxia
  • 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 0.0058% · 94 / 1,612,232 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.0075%

Highest in European (non-Finnish): AF 0.0075% (88 of 1,179,828 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)0.0075%88 / 1,179,8280~1 in 6700
African / African American0.0040%3 / 74,9480~1 in 12490
Remaining individuals0.0032%2 / 62,4440~1 in 15610
Admixed American · under-sampled0.0017%1 / 60,0020

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 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