RareResearch.AI
← Back to atlas

T699M

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
ThreonineMethionine at position 699 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Threonine → Methionine at position 699 in lumenal domain. ClinVar Conflicting including monogenic hearing loss. AlphaMissense 0.603, ΔΔG +0.01 (neutral). Same position as T699P (Atlas card).

Interactive 3D Structure

Wild-type reference
Wild-type T699 — hydrogen bond to F825
Fullscreen ↗
DynaMut2 mutant · T699M
Mutant M699 — energy-minimized; local contact network preserved
Fullscreen ↗

Bond changes · DynaMut2 interaction analysis

0 lost0 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondF825F825Preserved
Polar contactF825F825Preserved

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
0.01kcal/mol
Stabilising — mild
AlphaMissense
0.603
LPath
AlphaFold pLDDT
89
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable
ΔΔG confidence: Standard confidence · Lumenal

This variant is in a water-facing region, which is the regime the stability predictor was built and benchmarked for. Ordinary predictor error still applies.

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsMonogenic hearing loss
InheritanceMonogenic hearing loss.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00034%
cDNA changec.2096C>T
ClinVar accessionVCV000004522
Last evaluated2025/09/10 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Hearing loss, inheritance unstated (inheritance not specified).

  • Hearing loss, inheritance unstatedinheritance not specifiedsubmitted as: Monogenic hearing loss
  • 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 0.00034% · 5 / 1,460,530 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.00027%

Highest in European (non-Finnish): AF 0.00027% (3 of 1,111,822 alleles), in line with the global figure.

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.
Remaining individuals · under-sampled0.0017%1 / 60,3660
South Asian · under-sampled0.0012%1 / 86,2540
European (non-Finnish)0.00027%3 / 1,111,8220~1 in 185300

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 699 same neighbors as T699P: TRP700 (2.4 Å — Cat 2 outlier W700S region), VAL698 (2.5 Å), PHE825 (3.4 Å — W700-F825 π-stacking partner), SER826 (4.3 Å).

T699M is the second pathogenic substitution at 699 (with T699P). Where T699P introduced a backbone kink, T699M conservatively swaps small polar for small hydrophobic. Both perturb the W700-F825 π-stacking geometry that pulls W700S into Cat 2 destabilization.

ΔΔG neutral; AM 0.603 + monogenic hearing loss confirm severe consequence.

Amino-acid chemistry
Threonine (T) → Methionine (M) — small polar hydroxyl replaced by flexible sulfur-containing hydrophobic.
Position in the protein
C-terminal lumenal domain · position 699 (pLDDT 89). Same as T699P.

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG ≈ 0. AlphaMissense 0.603 confirms severe consequence.

Mechanism: W700-F825 π-stacking perturbation via T699 contact. Therapeutic: same W700-F825 microregion as T699P, W700C, W700S.

Why this matters

T699M + T699P + W700C + W700S — four Atlas variants converge on the W700-F825 π-stacking interface.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the T699M 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 T699M PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal
Natural variant699699 · in DFNA6; dbSNP:rs28937894