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H407Y

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
HistidineTyrosine at position 407 · TM3 (402-422), helical transmembrane · WFS1 (Wolframin)

His→Tyr p407 TM3 AM=0.10 ddg=+1.05 pLDDT=90. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type H407 — ionic bond to E403
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DynaMut2 mutant · H407Y
Mutant Y407 — ionic bond to E403 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost5 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondE403Lost
Hydrogen bondE403E403Preserved
Hydrogen bondL410Gained
Hydrogen bondS411S411Preserved
Hydrogen bondK634Gained
Polar contactE403E403Preserved
Polar contactP404P404Preserved
Polar contactL410Gained
Polar contactS411S411Preserved
Van der WaalsC360Lost
Van der WaalsE403E403Preserved
HydrophobicE403Gained
HydrophobicV633Lost
HydrophobicK634Gained
HydrophobicL637L637Preserved

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
1.05kcal/mol
Stabilising — moderate
AlphaMissense
0.100
LBen
AlphaFold pLDDT
90
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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0011%
cDNA changec.1219C>T
ClinVar accessionVCV000166583
Last evaluated2025/12/10 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

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

  • 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
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1; Wolfram syndrome 1
  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • 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 0.0011% · 17 / 1,614,070 alleles
Homozygotes
0
Highest-frequency population
African / African American · AF 0.020%

Highest in African / African American: AF 0.020% (15 of 75,060 alleles), 19.0x 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.
African / African American0.020%15 / 75,0600~1 in 2500
South Asian · under-sampled0.0011%1 / 91,0840
European (non-Finnish) · under-sampled0.000085%1 / 1,180,0320

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 analysis: PHE408 (2.5 Å — F408 TM3-TM7 interface!), ALA406 (2.5 Å), PRO404 (3.8 Å). Aromatic-rich TM3 environment near F408 cross-helix hub. The Atlas's neighbor extraction surfaces this variant's contacts.

Amino-acid chemistry
aromatic substitution stabilising
Position in the protein
TM3 (402-422)

Druggability Assessment

Cat 4 stabilising — see structural prose. AlphaMissense below threshold (AM under-call class) but mechanism is structurally clear from neighbor analysis. Therapeutic strategy: site-directed at the contacts identified above.

Why this matters

TM3 F408 interface variant.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the H407Y 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 H407Y PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane402422 · Helical