HDL2_JPH3

Gene
JPH3
Disease
HDL2
Inheritance
AD
Classification
Definitive
Total Score
15
Publications Reviewed
5
Publication Span
16.13 years
Last Updated
06/04/2025
Curator(s)
Laurel Hiatt, Harriet Dashnow

Genetic evidence

Total: 9

Singular EvidenceProbandsPMID:28339400669 patients from multiple studies
Collective EvidenceAllelePMID:283394002Negative correlation between repeat length and age of onset. Paper is a review review describing all cased in literature up to 2026
Collective EvidenceSegregationPMID:401870261Founder mutation identified
3 rows

Experimental evidence

Total: 6

ModelsNon-human model organismPMID:215550702HDL2 mouse model showed polyQ-mediated pathogenesis mechanism
ModelsNon-human model organismPMID:292086312Drosophila model
FunctionRegulatory ImpactPMID:116948762Splicing impact
3 rows

Note: Maximum score caps apply at evidence type, category, and supercategory levels, so section totals may be lower than the raw sum of row scores.