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P19L

Category 5 — IDR ExclusionConflictingCytoplasmic · predictedEditorial
ProlineLeucine at position 19 · N-terminal intrinsically disordered region (1-86) · WFS1 (Wolframin)

Pro→Leu p19 IDR AM=0.07 ddg=-0.3 pLDDT=34. ClinVar Conflicting evidence. Atlas mechanism: see structural analysis.

Interactive 3D Structure

Wild-type reference
Wild-type P19 — native residue, no strong sidechain contacts
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DynaMut2 mutant · P19L
Mutant L19 — energy-minimized; local contact network preserved
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Computational Predictions

DynaMut2 ΔΔG
-0.30kcal/mol
Destabilising — mild
AlphaMissense
0.066
LBen
AlphaFold pLDDT
34
model confidence
Schema
Cat 5
Category 5 — IDR Exclusion
ΔΔG confidence: Reduced confidence · Cytoplasmic
  • pLDDT 33.66 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 0.0077%
cDNA changec.56C>T
ClinVar accessionVCV000215372
Last evaluated2025/10/20 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

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

  • Cataract 41autosomal dominant · OMIM 116400submitted as: Cataract 41
  • 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
  • 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 0.0077% · 122 / 1,580,900 alleles
Homozygotes
0
Highest-frequency population
African / African American · AF 0.012%

Highest in African / African American: AF 0.012% (9 of 74,400 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.
African / African American0.012%9 / 74,4000~1 in 4130
European (non-Finnish)0.0090%105 / 1,164,2600~1 in 5540
Remaining individuals0.0065%4 / 61,2420~1 in 7660
South Asian0.0023%2 / 86,2760~1 in 21570
East Asian · under-sampled0.0023%1 / 43,3460
Admixed American · under-sampled0.0018%1 / 55,1700

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: GLN20 (2.4 Å), ALA18 (2.5 Å), PRO17 (4.5 Å). pLDDT 34 deep IDR. The Atlas's neighbor extraction surfaces this variant's contacts and connects them to the broader multi-variant target landscape.

Amino-acid chemistry
proline removal
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

Deep IDR proline removal.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the P19L 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 P19L PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A
Region186 · Disordered
Compositional bias1020 · Pro residues