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A684G

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
AlanineGlycine at position 684 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Alanine → Glycine at position 684 in lumenal domain. ClinVar Conflicting including type 2 diabetes. AlphaMissense 0.808, ΔΔG -0.38. THIRD substitution at position 684 (with A684T, A684V).

Interactive 3D Structure

Wild-type reference
Wild-type A684 — hydrogen bond to I688
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DynaMut2 mutant · A684G
Mutant G684 — van der waals contact to I688 lost
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Bond changes · DynaMut2 interaction analysis

0 lost0 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondQ687Q687Preserved
Hydrogen bondI688I688Preserved
Polar contactN682N682Preserved
Polar contactQ687Q687Preserved
Polar contactI688I688Preserved
Van der WaalsN682N682Preserved
Van der WaalsI688I688Preserved

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
-0.38kcal/mol
Destabilising — mild
AlphaMissense
0.808
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 conditionsType 2 diabetes mellitus
InheritanceType 2 diabetes documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0015%
cDNA changec.2051C>G
ClinVar accessionVCV000930623
Last evaluated2023/12/18 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant). These are dominant WFS1-related disorders and are clinically distinct from classic recessive Wolfram syndrome. This classification is not a statement about Wolfram syndrome severity.

  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • 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 0.0015% · 24 / 1,612,818 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.0019%

Highest in European (non-Finnish): AF 0.0019% (23 of 1,179,952 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)0.0019%23 / 1,179,9520~1 in 25650
Remaining individuals · under-sampled0.0016%1 / 62,4640

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 684 same neighbors as A684T/V: MET683 (2.5 Å), ARG685 (2.5 Å — R685P), GLN687 (4.0 Å — Q687H), ASN682 (4.0 Å), THR686 (4.4 Å).

A684G is the third substitution at position 684 — eliminating side chain entirely, introducing glycine backbone flexibility. The variant fold may shift more substantially than A684T's hydroxyl-introduction or A684V's volume-increase because of the backbone freedom.

ΔΔG 0.38 + AM 0.808 + T2D confirm severe consequence.

Amino-acid chemistry
Alanine (A) → Glycine (G) — small methyl-bearing residue replaced by smallest amino acid. Loss of side chain entirely.
Position in the protein
C-terminal lumenal domain · position 684 (pLDDT 88). Same as A684T, A684V.

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.38. AlphaMissense 0.808 + T2D confirm severe consequence.

Mechanism: backbone-flexibility introduction in the R685 microregion. Therapeutic: same A684 cluster.

Why this matters

A684G is the FOURTH variant at position 684 (with A684T, A684V, and adjacent R685P, Q687H, I688F). The 684-688 cluster is one of the densest Atlas hubs.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the A684G 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 A684G PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant684684 · in WFSL; greatly reduces protein expression compared to wild-type; dbSNP:rs387906930