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Y508C

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
TyrosineCysteine at position 508 · TM6 (496-516), helical transmembrane · WFS1 (Wolframin)

Tyrosine → Cysteine at position 508 inside TM6. ClinVar Conflicting including Wolfram. AlphaMissense 0.419 (below threshold), ΔΔG -1.22.

Interactive 3D Structure

Wild-type reference
Wild-type Y508 — hydrogen bond to P504
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DynaMut2 mutant · Y508C
Mutant C508 — polar contact to Y510 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost2 gained10 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondP504P504Preserved
Hydrogen bondC505C505Preserved
Hydrogen bondL511L511Preserved
Hydrogen bondL512L512Preserved
Polar contactP504P504Preserved
Polar contactC505C505Preserved
Polar contactY510Lost
Polar contactL511L511Preserved
Polar contactL512L512Preserved
Polar contactF882Lost
Van der WaalsL506Gained
Van der WaalsY510Y510Preserved
Van der WaalsL511Lost
HydrophobicL512Lost
HydrophobicP885Gained
HydrophobicF886F886Preserved

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
-1.22kcal/mol
Destabilising — moderate
AlphaMissense
0.419
Amb
AlphaFold pLDDT
85
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00012%
cDNA changec.1523A>G
ClinVar accessionVCV001027491
Last evaluated2024/01/17 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 508 in TM6. Neighbors: LEU507 (2.5 Å), VAL509 (2.5 Å), CYS505 (3.7 Å — C505Y region!), PRO504 (3.8 Å — P504L). The C505 contact at 3.7 Å is structurally significant.

Replacing Y508 with cysteine creates a potential new cysteine pair with the adjacent C505. The wild-type Y508 + C505 microregion now becomes a C505 + C508 cluster — two cysteines within 3.7 Å, geometrically capable of forming a new disulfide. This is potentially structurally disruptive.

|ΔΔG| 1.22 + AM 0.419 below threshold + Wolfram 1 confirm pathogenicity.

Amino-acid chemistry
Tyrosine (Y) → Cysteine (C) — large aromatic phenol replaced by small thiol.
Position in the protein
TM6 (residues 496–516) · position 508 (pLDDT 85).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 1.22 substantial. AlphaMissense 0.419 below threshold but Wolfram 1 + substantial ΔΔG confirm pathogenicity.

Mechanism: aberrant C505-C508 disulfide creation + lost Y508 aromatic packing. Therapeutic: TM6 P504-C505-Y508 microregion.

Why this matters

Y508C joins the TM6 cluster (P504L, C505Y). Three Atlas variants at three consecutive positions in TM6.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the Y508C 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 Y508C PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane496516 · Helical
Natural variant508512 · in WFS1