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G695S

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
GlycineSerine at position 695 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Glycine → Serine at position 695 in lumenal domain. ClinVar Conflicting including Cataract 41 and DFNA6. AlphaMissense 0.906, ΔΔG -0.90. Same position as G695D (Atlas card adjacent).

Interactive 3D Structure

Wild-type reference
Wild-type G695 — hydrogen bond to Y660
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DynaMut2 mutant · G695S
Mutant S695 — van der waals contact to Y660 lost
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Bond changes · DynaMut2 interaction analysis

1 lost0 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondV659V659Preserved
Hydrogen bondY660Y660Preserved
Hydrogen bondL829L829Preserved
Polar contactV659V659Preserved
Polar contactY660Y660Preserved
Polar contactL829L829Preserved
Van der WaalsV659V659Preserved
Van der WaalsY660Lost

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
-0.90kcal/mol
Destabilising — mild
AlphaMissense
0.906
LPath
AlphaFold pLDDT
82
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1; Cataract 41; Autosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceBoth AD (DFNA6, Cataract 41) and AR documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00034%
cDNA changec.2083G>A
ClinVar accessionVCV002630486
Last evaluated2025/07/16 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 695 same neighbors as G695D: GLU694 (2.5 Å), HIS696 (2.5 Å), LEU829 (4.0 Å — partner of L829P), ILE828 (4.0 Å), LEU693 (4.5 Å).

G695S is the second substitution at position 695 (with G695D). The serine introduces polarity rather than charge, but both substitutions disrupt the wild-type glycine's backbone-flexibility role and perturb the long-range contact with L829.

|ΔΔG| 0.90 + AlphaMissense 0.906 + Cataract 41 + DFNA6 confirm severe multi-tissue consequence.

Amino-acid chemistry
Glycine (G) → Serine (S) — smallest replaced by small polar hydroxyl. Loss of backbone flexibility, gain of H-bond.
Position in the protein
C-terminal lumenal domain · position 695 (pLDDT 82). Same as G695D.

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.90 — fold survives. AlphaMissense 0.906 + three phenotypes confirm severe consequence.

Mechanism: glycine removal + perturbed L829 long-range contact. Therapeutic: same target as G695D.

Why this matters

G695S + G695D at same position; both connect to L829P long-range. Position 695 is structurally critical for the L829-H696 microregion.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the G695S 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 G695S PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant695695 · in WFS1; dbSNP:rs28937891