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Y650C

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
TyrosineCysteine at position 650 · TM10 (632-652), helical transmembrane · WFS1 (Wolframin)

Tyrosine → Cysteine at position 650 inside TM10. ClinVar Conflicting including Cataract 41 + DFNA6. AlphaMissense 0.399 (below threshold), ΔΔG +0.31. Third Atlas variant at position 650 (with Y650H, Y650D).

Interactive 3D Structure

Wild-type reference
Wild-type Y650 — hydrogen bond to C647
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DynaMut2 mutant · Y650C
Mutant C650 — hydrogen bond to I338 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost1 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondI338Lost
Hydrogen bondF646F646Preserved
Hydrogen bondC647C647Preserved
Hydrogen bondR653Lost
Hydrogen bondS654S654Preserved
Polar contactF646F646Preserved
Polar contactC647C647Preserved
Polar contactW648W648Preserved
Polar contactY652Lost
Polar contactR653R653Preserved
Polar contactS654S654Preserved
Van der WaalsF646Gained
Van der WaalsW648Lost

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
0.31kcal/mol
Stabilising — mild
AlphaMissense
0.399
Amb
AlphaFold pLDDT
69
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Low confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins
  • pLDDT 68.69 below 70

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.

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 conditionsCataract 41; Autosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceAD: Cataract + DFNA6.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0042%
cDNA changec.1949A>G
ClinVar accessionVCV001218633
Last evaluated2024/10/24 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Cataract 41 (autosomal dominant, OMIM 116400); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); WFS1-related diabetes (autosomal dominant); Wolfram-like syndrome (autosomal dominant, OMIM 614296). 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.

  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • WFS1-related diabetesautosomal dominantsubmitted as: Type 2 diabetes mellitus
  • Wolfram-like syndromeautosomal dominant · OMIM 614296submitted as: Wolfram-like syndrome
  • 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 0.0042% · 68 / 1,613,672 alleles
Homozygotes
0
Highest-frequency population
East Asian · AF 0.016%

Highest in East Asian: AF 0.016% (7 of 44,864 alleles), 3.7x the global figure. The global AF describes the general population, not the at-risk group.

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.
East Asian0.016%7 / 44,8640~1 in 3200
European (non-Finnish)0.0047%55 / 1,180,0000~1 in 10730
Ashkenazi Jewish · under-sampled0.0034%1 / 29,6060
African / African American0.0027%2 / 74,8940~1 in 18720
South Asian0.0022%2 / 91,0780~1 in 22770
Admixed American · under-sampled0.0017%1 / 59,9960

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 650 same neighbors as Y650H/Y650D: PHE649 (2.5 Å), VAL651 (2.5 Å), CYS647 (3.7 Å — C647 already in close contact!), PHE646 (3.7 Å). The C647 contact suggests a potential Y650C-C647 disulfide formation.

Y650C is the THIRD substitution at position 650. The new C650 could potentially form a disulfide with C647 (3.7 Å away) — possibly a beneficial or aberrant chemistry depending on the wild-type C647 state. Compare with Y650H (preserved aromatic) and Y650D (charge introduction).

AM 0.399 below threshold; multi-phenotype confirms.

Amino-acid chemistry
Tyrosine (Y) → Cysteine (C) — aromatic phenol replaced by thiol. Loss of aromatic + introduction of disulfide-capable thiol.
Position in the protein
TM10 (residues 632–652) · position 650 (pLDDT 69 borderline).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted (AM under-call). ΔΔG +0.31. AlphaMissense 0.399 below threshold and multi-phenotype does not resolve it (ClinVar: conflicting submissions).

Mechanism: aromatic loss + potential aberrant disulfide with C647. Therapeutic: same Y650 microregion as Y650H/Y650D.

Why this matters

Y650C completes the Y650 three-substitution series (Y650H, Y650D, Y650C) — position 650 is a multi-substitution hotspot in TM10.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the Y650C 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 Y650C PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane632652 · Helical