PRINS

Chr 10ARAD

psoriasis associated non-protein coding RNA induced by stress

Also known as: NCRNA00074

PRINS encodes an enzyme involved in purine nucleotide metabolism that is essential for maintaining adequate ATP levels in red blood cells. Mutations cause congenital nonspherocytic hemolytic anemia type 7, which presents as chronic hemolysis due to red blood cell enzyme deficiency, and may also contribute to myocardial infarction susceptibility. The condition follows autosomal recessive inheritance for the anemia phenotype, though some variants may show autosomal dominant effects.

Summary from OMIM
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Primary Disease Associations & Inheritance

{Myocardial infarction, susceptibility to}MIM #608446
Anemia, congenital, nonspherocytic hemolytic, 7MIM #230450
AR
Anemia, congenital, nonspherocytic hemolytic, 7MIM #230450
AR
[Adenosine triphosphate, elevated, of erythrocytes]MIM #102900
AD
1
Active trials
30
Pubs (1 yr)
10
P/LP submissions
P/LP missense
LOEUF
Mechanism
Clinical SummaryPRINS
📋
ClinVar Variants
10 unique Pathogenic / Likely Pathogenic· 1 VUS of 14 total submissions
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
📖
GeneReview available — PRINS
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/PRINS?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

14 submitted variants in ClinVar

Classification Summary

Pathogenic9
Likely Pathogenic1
VUS1
Likely Benign1
Benign1
9
Pathogenic
1
Likely Pathogenic
1
VUS
1
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
9
Likely Pathogenic
1
VUS
1
Likely Benign
1
Benign
1
Total13

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PRINS · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Literature
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗