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S430W

Category 3/4 — Most DruggableLikely pathogenicTransmembrane · predictedEditorial
SerineTryptophan at position 430 · TM4 (427-447), helical transmembrane · WFS1 (Wolframin)

Serine → Tryptophan at position 430 inside TM4. ClinVar Likely pathogenic. AlphaMissense 0.979, DynaMut2 ΔΔG -0.74 kcal/mol (destabilising). A massive volume increase in a TM helix.

Interactive 3D Structure

Wild-type reference
Wild-type S430 — hydrogen bond to V434
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DynaMut2 mutant · S430W
Mutant W430 — polar contact contact to A559 lost
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Bond changes · DynaMut2 interaction analysis

1 lost7 gained5 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondA433Gained
Hydrogen bondV434V434Preserved
Hydrogen bondT552Gained
Hydrogen bondG555G555Preserved
Polar contactP428P428Preserved
Polar contactV434V434Preserved
Polar contactT552Gained
Polar contactA559Lost
Van der WaalsP428P428Preserved
Van der WaalsT552Gained
Van der WaalsR558Gained
HydrophobicP428Gained
HydrophobicA559Gained

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
-0.74kcal/mol
Destabilising — mild
AlphaMissense
0.979
LPath
AlphaFold pLDDT
90
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationLikely pathogenic
Review statusno assertion criteria provided
Associated conditions(no specific conditions catalogued for S430W)
InheritanceInheritance not specified. ClinVar Likely pathogenic.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00014%
cDNA changec.1289C>G
ClinVar accessionVCV001297548
Last evaluated1/01/01 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 430 sits near the start of TM4. The AlphaFold model places S430 within 5 Å of CYS429 (2.5 Å), GLU431 (2.5 Å — same E431 contacted by A559D and P428R), SER551 (4.0 Å — TM4-TM7 cross-helix), PRO428 (4.1 Å), and ALA433 (4.4 Å). The E431 contact at 2.5 Å is structurally significant — the wild-type serine's hydroxyl likely H-bonds to E431's carboxylate.

Replacing serine with tryptophan introduces a massive volume increase. The pocket sized for serine cannot accommodate tryptophan without substantial rearrangement. The H-bond to E431 is lost (tryptophan's indole is aromatic, not H-bond-donating in this geometry). The cross-helix contact to S551 in TM7 is perturbed.

The |ΔΔG| of 0.74 reflects fold absorption at meaningful cost. AlphaMissense's 0.979 confirms severe functional consequence.

Amino-acid chemistry
Serine (S) → Tryptophan (W) — small polar hydroxyl replaced by bulky aromatic indole. Roughly four-fold side-chain volume increase plus aromatic π-system added.
Position in the protein
TM4 (residues 427–447) · position 430 near the start of TM4 (pLDDT 90).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.74 kcal/mol — fold survives. AlphaMissense 0.979 confirms severe functional consequence.

The mechanism is volume mismatch in TM4 plus loss of the S430-E431 H-bond. Therapeutic strategy: site-directed at the E431 microregion (also touched by A559D and P428R).

Why this matters

S430W is the fourth Atlas variant contacting E431 (with A559D, P428R, E431Q in the next card). E431 emerges as a hub residue in the WFS1 lumenal-membrane interface — multiple pathogenic variants converge on it.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the S430W 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 S430W PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane427447 · Helical