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K876T

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
LysineThreonine at position 876 · TM11 (870-890), helical transmembrane · WFS1 (Wolframin)

Lysine → Threonine at position 876 inside TM11. ClinVar Conflicting including T2D. AlphaMissense 0.29 (below threshold) — AM under-call. DynaMut2 ΔΔG -0.49.

Interactive 3D Structure

Wild-type reference
Wild-type K876 — hydrogen bond to D880
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DynaMut2 mutant · K876T
Mutant T876 — hydrogen bond to A890 lost (7 contacts lost)
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Bond changes · DynaMut2 interaction analysis

7 lost0 gained9 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondH872H872Preserved
Hydrogen bondG873G873Preserved
Hydrogen bondF879F879Preserved
Hydrogen bondD880D880Preserved
Hydrogen bondA889Lost
Hydrogen bondA890Lost
Polar contactH872H872Preserved
Polar contactG873G873Preserved
Polar contactA878Lost
Polar contactF879F879Preserved
Polar contactD880D880Preserved
Polar contactA889Lost
Polar contactA890Lost
Van der WaalsH872Lost
Van der WaalsA878Lost
HydrophobicA889A889Preserved

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
-0.49kcal/mol
Destabilising — mild
AlphaMissense
0.287
LBen
AlphaFold pLDDT
84
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 conditionsType 2 diabetes mellitus
InheritanceT2D documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0088%
cDNA changec.2627A>C
ClinVar accessionVCV000281647
Last evaluated2025/08/22 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant). These are dominant WFS1-related disorders and are clinically distinct from classic recessive Wolfram syndrome. This classification is not a statement about Wolfram syndrome severity.

  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • 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 0.0088% · 142 / 1,613,230 alleles
Homozygotes
1
Highest-frequency population
African / African American · AF 0.174%

Highest in African / African American: AF 0.174% (131 of 75,078 alleles), 19.8x the global figure. The global AF describes the general population, not the at-risk group.

1 homozygote reported in gnomAD v4 (1 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 American0.174%131 / 75,0781~1 in 290
Remaining individuals0.0064%4 / 62,5000~1 in 7810
Admixed American0.0050%3 / 60,0320~1 in 10010
European (non-Finnish)0.00034%4 / 1,180,0420~1 in 147510

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 876 in TM11. Neighbors: PHE877 (2.4 Å), VAL875 (2.5 Å — partner of V875M), HIS872 (3.7 Å — same H872 in V871G cluster).

K876T joins the TM11 multi-variant cluster. The wild-type K876 likely serves as a 'positive-inside rule' anchor at the TM11 cytoplasmic end; losing it perturbs TM11 topology. AM 0.29 under-call; T2D does not resolve it (ClinVar: conflicting submissions).

Amino-acid chemistry
Lysine (K) → Threonine (T) — long positively-charged amine replaced by small polar hydroxyl. Loss of charge.
Position in the protein
TM11 (residues 870–890) · position 876 (pLDDT 84).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.49. AlphaMissense 0.29 below threshold and T2D does not resolve it (ClinVar: conflicting submissions).

Mechanism: loss of positive-inside anchor at TM11. Therapeutic: TM11 multi-variant cluster (V871G/M, V875M, A874T, P885L).

Why this matters

K876T is the 7th TM11 cluster variant — the helix is one of the most variant-dense in the Atlas.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the K876T 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 K876T PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane870890 · Helical