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I561V

Category 4 — Stable Fold, Function DisruptedConflictingCytoplasmic · predictedEditorial
IsoleucineValine at position 561 · Connecting loop · WFS1 (Wolframin)

Ile→Val p561 loop AM=0.06 ddg=-0.25 pLDDT=85. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type I561 — hydrogen bond to L565
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DynaMut2 mutant · I561V
Mutant V561 — polar contact contact to L557 lost
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Bond changes · DynaMut2 interaction analysis

1 lost0 gained7 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondL557L557Preserved
Hydrogen bondL565L565Preserved
Polar contactL557L557Preserved
Polar contactA559A559Preserved
Polar contactF564Lost
Polar contactL565L565Preserved
Van der WaalsL557L557Preserved
HydrophobicL557L557Preserved

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
-0.25kcal/mol
Destabilising — mild
AlphaMissense
0.055
LBen
AlphaFold pLDDT
85
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted
ΔΔG confidence: Standard confidence · Cytoplasmic

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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0022%
cDNA changec.1681A>G
ClinVar accessionVCV001320392
Last evaluated2026/01/24 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity, but does not state what it is classified for. ClinVar records the condition only as "Inborn genetic diseases", "not specified", "not provided". A classification without a phenotype and an inheritance mode cannot be read as a statement about Wolfram syndrome risk or severity.

  • 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 0.0022% · 36 / 1,612,894 alleles
Homozygotes
0
Highest-frequency population
East Asian · AF 0.042%

Highest in East Asian: AF 0.042% (19 of 44,892 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.
East Asian0.042%19 / 44,8920~1 in 1180
Remaining individuals0.022%14 / 62,4760~1 in 2230
Admixed American · under-sampled0.0017%1 / 60,0040
South Asian · under-sampled0.0011%1 / 91,0780
European (non-Finnish) · under-sampled0.000085%1 / 1,180,0360

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: GLY562 (2.5 Å — G562 in A559D region!), SER560 (2.5 Å — same S560 as A559D!), LEU557 (3.7 Å — L557 in R558C loop!). Same R558C connecting loop. The Atlas's neighbor extraction surfaces this variant's contacts and connects them to the broader multi-variant target landscape.

Amino-acid chemistry
conservative volume reduction
Position in the protein
Connecting loop

Druggability Assessment

Cat 4 — see structural prose. AlphaMissense below threshold (AM under-call class) but mechanism is structurally identified. Therapeutic strategy: site-directed at contacts identified above, or wet-lab validation if pLDDT borderline/below 50.

Why this matters

R558C connecting loop variant.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the I561V 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 I561V PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin