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R587Q

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
ArginineGlutamine at position 587 · Connecting loop · WFS1 (Wolframin)

Arg→Gln p587 loop AM=0.08 ddg=-0.05 pLDDT=77. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type R587 — hydrogen bond to L583
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DynaMut2 mutant · R587Q
Mutant Q587 — hydrogen bond contact to T590 lost
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Bond changes · DynaMut2 interaction analysis

0 lost0 gained8 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondL583L583Preserved
Hydrogen bondT590T590Preserved
Hydrogen bondS591S591Preserved
Polar contactL583L583Preserved
Polar contactQ584Q584Preserved
Polar contactT590T590Preserved
Polar contactS591S591Preserved
Van der WaalsS591S591Preserved

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
-0.05kcal/mol
Destabilising — mild
AlphaMissense
0.081
LBen
AlphaFold pLDDT
77
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)Low frequency · AF 0.028%
cDNA changec.1760G>A
ClinVar accessionVCV000215361
Last evaluated2025/12/30 00:00

Observed in the general population.

Classified for1★ unverified submission

ClinVar classifies this variant as Conflicting classifications of pathogenicity for WFS1-related diabetes (autosomal dominant); Wolfram syndrome (classic) (autosomal recessive, OMIM 222300); Optic atrophy / optic neuropathy (autosomal dominant); WFS1-related spectrum (unresolved mode) (dominant or recessive); Nonsyndromic hearing loss (DFNA6/14/38) (autosomal dominant, OMIM 600965). 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.

  • WFS1-related diabetesautosomal dominantsubmitted as: Monogenic diabetes; Type 2 diabetes mellitus
  • Wolfram syndrome (classic)autosomal recessive · OMIM 222300submitted as: Wolfram syndrome 1
  • Optic atrophy / optic neuropathyautosomal dominantsubmitted as: Optic atrophy
  • WFS1-related spectrum (unresolved mode)dominant or recessivesubmitted as: WFS1-Related Spectrum Disorders
  • Nonsyndromic hearing loss (DFNA6/14/38)autosomal dominant · OMIM 600965submitted as: Autosomal dominant nonsyndromic hearing loss 6
  • 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 0.028% · 458 / 1,614,076 alleles
Homozygotes
0
Highest-frequency population
Ashkenazi Jewish · AF 0.439%

Highest in Ashkenazi Jewish: AF 0.439% (130 of 29,608 alleles), 15.5x 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.
Ashkenazi Jewish0.439%130 / 29,6080~1 in 110
Middle Eastern0.231%14 / 6,0620~1 in 220
Admixed American0.188%113 / 60,0300~1 in 270
Remaining individuals0.082%51 / 62,5100~1 in 610
African / African American0.013%10 / 75,0520~1 in 3750
European (non-Finnish)0.011%128 / 1,180,0380~1 in 4610
East Asian0.0089%4 / 44,8640~1 in 5610
South Asian0.0088%8 / 91,0860~1 in 5690

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: TRP588 (2.4 Å), ALA586 (2.5 Å — same loop as R587W), THR590 (3.5 Å). Same position as R587W. 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
Connecting loop

Druggability Assessment

Cat 3/4 — 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

R587W + R587Q sister variants.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the R587Q 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 R587Q PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin