PRIM1

Chr 12AR

DNA primase subunit 1

Also known as: PDIL, p49

The replication of DNA in eukaryotic cells is carried out by a complex chromosomal replication apparatus, in which DNA polymerase alpha and primase are two key enzymatic components. Primase, which is a heterodimer of a small subunit and a large subunit, synthesizes small RNA primers for the Okazaki fragments made during discontinuous DNA replication. The protein encoded by this gene is the small, 49 kDa primase subunit. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.691 OMIM phenotype
Clinical SummaryPRIM1
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Gene-Disease Validity (ClinGen)
primordial dwarfism-immunodeficiency-lipodystrophy syndrome · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.69LOEUF
pLI 0.001
Z-score 2.67
OE 0.40 (0.240.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
2.05Z-score
OE missense 0.60 (0.520.69)
122 obs / 204.3 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.40 (0.240.69)
00.351.4
Missense OE?0.60 (0.520.69)
00.61.4
Synonymous OE?0.88
01.21.6
LoF obs/exp: 9 / 22.8Missense obs/exp: 122 / 204.3Syn Z: 0.74
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongPRIM1-related primordial dwarfismLOFAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6358th %ile
GOF
0.2995th %ile
LOF
0.3356th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PRIM1 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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