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R42Q

Category 5 — IDR ExclusionConflictingCytoplasmic · predictedEditorial
ArginineGlutamine at position 42 · N-terminal intrinsically disordered region (1-86) · WFS1 (Wolframin)

Arg→Gln p42 IDR AM=0.07 ddg=-0.05 pLDDT=29. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type R42 — native residue, no strong sidechain contacts
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DynaMut2 mutant · R42Q
Mutant Q42 — energy-minimized; local contact network preserved
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Computational Predictions

DynaMut2 ΔΔG
-0.05kcal/mol
Destabilising — mild
AlphaMissense
0.069
LBen
AlphaFold pLDDT
29
model confidence
Schema
Cat 5
Category 5 — IDR Exclusion
ΔΔG confidence: Reduced confidence · Cytoplasmic
  • pLDDT 28.84 below 70

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 conditions(no specific conditions catalogued)
InheritanceConflicting ClinVar classifications.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0011%
cDNA changec.125G>A
ClinVar accessionVCV000517412
Last evaluated2025/06/12 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for Wolfram syndrome (classic) (autosomal recessive, OMIM 222300). Classic Wolfram syndrome is recessive: a single copy does not produce it, and this classification says nothing about a carrier's own risk.

  • 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 0.0011% · 17 / 1,583,128 alleles
Homozygotes
0
Highest-frequency population
European (non-Finnish) · AF 0.0012%

Highest in European (non-Finnish): AF 0.0012% (14 of 1,165,088 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.
Middle Eastern · under-sampled0.017%1 / 6,0440
Admixed American · under-sampled0.0018%1 / 55,8900
Remaining individuals · under-sampled0.0016%1 / 61,3660
European (non-Finnish)0.0012%14 / 1,165,0880~1 in 41610

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: ALA43 (2.4 Å — A43V!), PRO41 (2.5 Å), ARG40 (4.8 Å). pLDDT 29 deep IDR. Adjacent to A43V. The Atlas's neighbor extraction surfaces this variant's contacts and connects them to the broader multi-variant target landscape.

Amino-acid chemistry
charge loss, amide preserved
Position in the protein
N-terminal IDR

Druggability Assessment

Cat 5 IDR — 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

IDR adjacent to A43V.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R42Q 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 R42Q PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A
Region186 · Disordered