MCM10

Chr 10AR

minichromosome maintenance 10 replication initiation factor

Also known as: CNA43, DNA43, IMD80, PRO2249

MCM10 encodes a replication initiation factor that brings together the MCM2-7 helicase and DNA polymerase alpha/primase complex to initiate DNA replication and prevent replication-associated DNA damage. Autosomal recessive mutations cause immunodeficiency 80 with or without cardiomyopathy, affecting immune system function and potentially cardiac muscle. The gene shows low constraint to loss-of-function variation (pLI near zero), consistent with its recessive inheritance pattern.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Immunodeficiency 80 with or without cardiomyopathyMIM #619313
AR
0
Active trials
25
Pubs (1 yr)
27
P/LP submissions
4%
P/LP missense
0.84
LOEUF
Mechanism
Clinical SummaryMCM10
🧬
Gene-Disease Validity (ClinGen)
immunodeficiency 80 with or without congenital cardiomyopathy · 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.
📋
ClinVar Variants
25 unique Pathogenic / Likely Pathogenic· 124 VUS of 216 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.84LOEUF
pLI 0.000
Z-score 2.48
OE 0.62 (0.470.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.17Z-score
OE missense 1.02 (0.951.10)
496 obs / 485.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.62 (0.470.84)
00.351.4
Missense OE1.02 (0.951.10)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 31 / 49.9Missense obs/exp: 496 / 485.6Syn Z: 0.73

ClinVar Variant Classifications

216 submitted variants in ClinVar

Classification Summary

Pathogenic24
Likely Pathogenic1
VUS124
Likely Benign22
Benign22
24
Pathogenic
1
Likely Pathogenic
124
VUS
22
Likely Benign
22
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
1
22
0
24
Likely Pathogenic
0
0
1
0
1
VUS
0
117
7
0
124
Likely Benign
0
13
1
8
22
Benign
0
6
13
3
22
Total11374411193

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

View in ClinVar →

Protein Context — Lollipop Plot

MCM10 · 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
Open Research Assistant →