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M306T

Category 4 — Stable Fold, Function DisruptedConflictingCytoplasmic · predictedEditorial
MethionineThreonine at position 306 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Methionine → Threonine at position 306 in N-terminal cytoplasmic domain. ClinVar Conflicting including monogenic diabetes + WFS1 spectrum. AlphaMissense 0.14 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.03 (neutral).

Interactive 3D Structure

Wild-type reference
Wild-type M306 — hydrogen bond to Y302
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DynaMut2 mutant · M306T
Mutant T306 — hydrogen bond to L303 lost (2 contacts lost)
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Bond changes · DynaMut2 interaction analysis

2 lost0 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondY302Y302Preserved
Hydrogen bondL303Lost
Hydrogen bondA310A310Preserved
Polar contactY302Y302Preserved
Polar contactL303L303Preserved
Polar contactI304I304Preserved
Polar contactA310A310Preserved
Van der WaalsY302Y302Preserved
Van der WaalsI304I304Preserved
HydrophobicY302Lost

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
Destabilising — mild
AlphaMissense
0.144
LBen
AlphaFold pLDDT
66
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Reduced confidence · Cytoplasmic
  • pLDDT 65.69 below 70

The underlying AlphaFold model has pLDDT below 70 at this position, so the structure the stability calculation was run on is itself uncertain.

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 conditionsMonogenic diabetes; WFS1-Related Spectrum Disorders
InheritanceMulti-phenotype.
Population frequency (gnomAD v4)Low frequency · AF 0.068%
cDNA changec.917T>C
ClinVar accessionVCV000167850
Last evaluated2026/01/27 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant); WFS1-related spectrum (unresolved mode) (dominant or recessive); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965). ClinVar records this under a WFS1-spectrum label that spans both disease families — recessive Wolfram syndrome (biallelic) and the dominant WFS1-related disorders. The label does not resolve which applies, and it is not a severity statement.

  • WFS1-related diabetesautosomal dominantsubmitted as: Monogenic diabetes
  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • 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
  • 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 0.068% · 1,102 / 1,614,058 alleles
Homozygotes
9
Highest-frequency population
African / African American · AF 1.33%

Highest in African / African American: AF 1.33% (998 of 74,986 alleles), 19.5x the global figure. The global AF describes the general population, not the at-risk group.

9 homozygotes reported in gnomAD v4 (9 African / African American). 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.
African / African American1.33%998 / 74,9869~1 in 38
Remaining individuals0.069%43 / 62,5040~1 in 730
Admixed American0.053%32 / 60,0140~1 in 940
South Asian0.0044%4 / 91,0800~1 in 11390
European (non-Finnish)0.0021%25 / 1,179,9800~1 in 23600

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 306 near TM1 boundary. Neighbors: ASP305 (2.5 Å), ALA307 (2.5 Å — partner of S308C!), LEU303 (3.9 Å).

M306T near the S308C variant region. Loss of methionine-specific chemistry + introduced polarity. AM 0.14 under-call; multi-phenotype confirms.

Amino-acid chemistry
Methionine (M) → Threonine (T) — flexible sulfur-containing hydrophobic replaced by small polar hydroxyl.
Position in the protein
N-terminal cytoplasmic domain · position 306 near TM1 boundary (pLDDT 66).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). ΔΔG ≈ 0. AlphaMissense 0.14 below threshold but multi-phenotype confirms.

Mechanism: lost methionine chemistry near TM1 boundary. Therapeutic: same 305-308 microregion as S308C.

Why this matters

M306T + S308C in same TM1-boundary region.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the M306T 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 M306T PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A