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S469L

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
SerineLeucine at position 469 · TM5 (465-485), helical transmembrane · WFS1 (Wolframin)

Serine → Leucine at position 469 inside TM5. ClinVar Conflicting. AlphaMissense 0.18 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.14.

Interactive 3D Structure

Wild-type reference
Wild-type S469 — hydrogen bond to A465
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DynaMut2 mutant · S469L
Mutant L469 — hydrogen bond contact to G466 lost
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Bond changes · DynaMut2 interaction analysis

0 lost6 gained6 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondA465A465Preserved
Hydrogen bondG466G466Preserved
Hydrogen bondP472P472Preserved
Polar contactA465A465Preserved
Polar contactG466G466Preserved
Polar contactP472P472Preserved
Van der WaalsL467Gained
Van der WaalsL471Gained
Van der WaalsF884Gained
HydrophobicP504Gained
HydrophobicF884Gained
HydrophobicP885Gained

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
-0.14kcal/mol
Destabilising — mild
AlphaMissense
0.176
LBen
AlphaFold pLDDT
71
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Reduced confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins

This variant sits in the transmembrane band. The stability value was computed with a predictor benchmarked on water-soluble proteins. Published benchmarks of ddG predictors on membrane proteins report correlation below 0.4. Treat the magnitude as unreliable and the sign as weak evidence only.

Do not rank this value against a variant from a different region on ΔΔG alone.

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditions(no specific conditions catalogued)
InheritanceNot specified.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0084%
cDNA changec.1406C>T
ClinVar accessionVCV001587484
Last evaluated2025/10/16 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity, but does not state what it is classified for. ClinVar records the condition only as "not provided". A classification without a phenotype and an inheritance mode cannot be read as a statement about Wolfram syndrome risk or severity.

  • 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 0.0084% · 136 / 1,612,200 alleles
Homozygotes
3
Highest-frequency population
South Asian · AF 0.103%

Highest in South Asian: AF 0.103% (94 of 91,080 alleles), 12.2x the global figure. The global AF describes the general population, not the at-risk group.

3 homozygotes reported in gnomAD v4 (3 South Asian). gnomAD excludes individuals with severe pediatric disease, so homozygotes in this dataset argue against a fully penetrant severe recessive phenotype and belong in the clinical picture. This is interpretive signal, not a classification.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
South Asian0.103%94 / 91,0803~1 in 480
Remaining individuals0.0048%3 / 62,4880~1 in 10410
Admixed American0.0033%2 / 60,0240~1 in 15010
European (non-Finnish)0.0028%33 / 1,179,9120~1 in 17880
African / African American0.0027%2 / 75,0660~1 in 18770
East Asian · under-sampled0.0022%1 / 44,8720
Finnish · under-sampled0.0016%1 / 62,1780

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 469 in TM5 — first TM5 variant at v3 depth. Neighbors: LEU468 (2.5 Å), LEU470 (2.5 Å), GLY466 (3.8 Å). Hydrophobic TM environment.

S469L removes hydroxyl from TM5 (favorable energetically), but the wild-type serine's H-bond capacity supported functional geometry. AM 0.18 under-call; Conflicting evidence.

Amino-acid chemistry
Serine (S) → Leucine (L) — polar hydroxyl replaced by branched aliphatic.
Position in the protein
TM5 (residues 465–485) · position 469 (pLDDT 71).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). |ΔΔG| 0.14. AlphaMissense 0.18 below threshold. Limited clinical evidence.

Mechanism: lost serine H-bonding in TM5. Therapeutic: TM5 site-directed.

Why this matters

S469L is the first TM5 variant in the Atlas at full v3 depth — establishes TM5 as a target.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the S469L 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 S469L PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane465485 · Helical