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T361S

Category 3/4 — Most DruggableConflictingTransmembrane · predictedEditorial
ThreonineSerine at position 361 · Connecting loop · WFS1 (Wolframin)

Threonine → Serine at position 361 in a connecting loop. ClinVar Conflicting including Wolfram + Wolfram-like. AlphaMissense 0.756, ΔΔG +0.03 (neutral). Same position as T361I (Atlas flagship pathogenic-stabilising card).

Interactive 3D Structure

Wild-type reference
Wild-type T361 — hydrogen bond to F365
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DynaMut2 mutant · T361S
Mutant S361 — hydrogen bond to V358 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost3 gained11 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondM357M357Preserved
Hydrogen bondV358V358Preserved
Hydrogen bondV364V364Preserved
Hydrogen bondF365F365Preserved
Hydrogen bondW540Gained
Polar contactM357M357Preserved
Polar contactV358V358Preserved
Polar contactI359I359Preserved
Polar contactK363K363Preserved
Polar contactV364V364Preserved
Polar contactF365F365Preserved
Polar contactW540Gained
Van der WaalsM357Lost
Van der WaalsI359I359Preserved
Van der WaalsK363Gained
HydrophobicF408Lost
HydrophobicV536Lost
HydrophobicW540Lost

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
0.03kcal/mol
Stabilising — mild
AlphaMissense
0.756
LPath
AlphaFold pLDDT
91
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable
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 conditionsWolfram-like syndrome; Wolfram syndrome 1
InheritanceAD and AR documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.000068%
cDNA changec.1082C>G
ClinVar accessionVCV001486730
Last evaluated2024/10/25 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram-like syndrome (autosomal dominant, OMIM 614296); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); WFS1-related diabetes (autosomal dominant); Cataract 41 (autosomal dominant, OMIM 116400). These are two clinically distinct disease families: classic Wolfram syndrome requires two affected copies, while the dominant WFS1-related disorders do not. One label covers both, and it does not tell you which applies to a given person.

  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • 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 0.000068% · 1 / 1,461,890 alleles
Homozygotes
0

Every ancestry group with observed alleles falls below the reporting threshold (min 2 alleles, min 500 sampled) — too sparse to name a highest-frequency population.

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.
Admixed American · under-sampled0.0022%1 / 44,7240

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 361 same neighbors as T361I: LEU362 (2.5 Å), CYS360 (2.5 Å), VAL358 (3.8 Å), MET357 (3.9 Å).

T361S is the most conservative substitution possible at position 361 — preserving the H-bonding hydroxyl while only removing the methyl group. ΔΔG essentially neutral. Yet AlphaMissense 0.756 + Wolfram + Wolfram-like confirm pathogenicity.

Mechanism is the same as T361I (Atlas card): functional disruption rather than fold disruption. The K363 H-bond partner geometry is fine-tuned by the wild-type threonine's specific methyl positioning that serine cannot replicate.

Amino-acid chemistry
Threonine (T) → Serine (S) — small polar hydroxyl with methyl replaced by small polar hydroxyl without methyl. Conservative.
Position in the protein
Connecting loop · position 361 (pLDDT 91). Same as T361I.

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG ≈ 0. AlphaMissense 0.756 + multi-syndrome confirm severe consequence.

Mechanism: fine-grained geometry disruption at the T361-K363 H-bond pair. Therapeutic: same target as T361I.

Why this matters

T361S + T361I at same position — both pathogenic by functional mechanism. Position 361 demonstrates that even the most conservative T→S substitution breaks function.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the T361S 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 T361S PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin