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L445V

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
LeucineValine at position 445 · TM4 (427-447), helical transmembrane · WFS1 (Wolframin)

Leu→Val p445 TM4 AM=0.10 ddg=+0.32 pLDDT=84. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type L445 — hydrogen bond to V441
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DynaMut2 mutant · L445V
Mutant V445 — hydrogen bond to V441 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost2 gained4 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondV441V441Preserved
Hydrogen bondT442Gained
Polar contactV441V441Preserved
Polar contactT442T442Preserved
Polar contactS443S443Preserved
Polar contactL447Gained
Van der WaalsV441Lost
Van der WaalsS443Lost
HydrophobicV441Lost

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
0.32kcal/mol
Stabilising — mild
AlphaMissense
0.096
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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0060%
cDNA changec.1333C>G
ClinVar accessionVCV000349318
Last evaluated2025/09/02 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 spectrum (unresolved mode) (dominant or recessive); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300). 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.

  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • 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 0.0060% · 97 / 1,613,344 alleles
Homozygotes
0
Highest-frequency population
Admixed American · AF 0.012%

Highest in Admixed American: AF 0.012% (7 of 60,002 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.
Admixed American0.012%7 / 60,0020~1 in 4290
European (non-Finnish)0.0076%90 / 1,180,0240~1 in 6560

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: SER446 (2.5 Å), TYR444 (2.5 Å — partner of S443R), THR442 (3.8 Å). Near S443R cluster. The Atlas's neighbor extraction surfaces this variant's contacts.

Amino-acid chemistry
conservative volume reduction
Position in the protein
TM4 (427-447)

Druggability Assessment

Cat 4 — 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

TM4 cluster adjacent to S443R.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the L445V 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 L445V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane427447 · Helical