PRIMPOL

Chr 4AD

primase and DNA directed polymerase

Also known as: CCDC111, MYP22, Primpol1

The protein is a DNA primase-polymerase that facilitates DNA replication fork progression by bypassing DNA damage lesions and reinitiating DNA synthesis in both nuclear and mitochondrial DNA. Mutations cause autosomal dominant myopia 22. The gene is not highly constrained against loss-of-function variants (LOEUF 1.328).

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Myopia 22, autosomal dominantMIM #615420
AD
Myopia 22, autosomal dominantMIM #615420
AD
0
Active trials
19
Pubs (1 yr)
105
P/LP submissions
0%
P/LP missense
1.33
LOEUF
Mechanism
Clinical SummaryPRIMPOL
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
105 unique Pathogenic / Likely Pathogenic· 102 VUS of 223 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.33LOEUF
pLI 0.000
Z-score 0.16
OE 0.97 (0.721.33)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.30Z-score
OE missense 1.05 (0.951.16)
297 obs / 282.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.97 (0.721.33)
00.351.4
Missense OE1.05 (0.951.16)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 28 / 28.9Missense obs/exp: 297 / 282.8Syn Z: 0.24

ClinVar Variant Classifications

223 submitted variants in ClinVar

Classification Summary

Pathogenic96
Likely Pathogenic9
VUS102
Likely Benign11
Benign5
96
Pathogenic
9
Likely Pathogenic
102
VUS
11
Likely Benign
5
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
96
0
96
Likely Pathogenic
0
0
9
0
9
VUS
2
85
15
0
102
Likely Benign
0
6
2
3
11
Benign
0
3
1
1
5
Total2941234223

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PRIMPOL · 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.

Search ClinicalTrials.gov →
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 ↗