MASP1

Chr 3

MBL associated serine protease 1

Also known as: 3MC1, CRARF, CRARF1, MAP-1, MAP1, MASP, MASP-3, MASP3

This gene encodes a serine protease that activates the lectin pathway of complement activation and may also function in coagulation by cleaving fibrinogen and factor XIII. Mutations cause 3MC syndrome 1, an autosomal recessive disorder characterized by multiple congenital anomalies affecting craniofacial development, skeletal system, and other organ systems. The gene shows very low constraint to loss-of-function variants, consistent with its recessive inheritance pattern.

ResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismLOEUF 0.98
Clinical SummaryMASP1
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Gene-Disease Validity (ClinGen)
3MC syndrome 1 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.98LOEUF
pLI 0.000
Z-score 1.66
OE 0.68 (0.490.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.16Z-score
OE missense 1.02 (0.941.11)
401 obs / 392.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.68 (0.490.98)
00.351.4
Missense OE1.02 (0.941.11)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 22 / 32.1Missense obs/exp: 401 / 392.1Syn Z: -1.55
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveMASP1-related 3MC syndromeLOFAR

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

DN
0.6453th %ile
GOF
0.7027th %ile
LOF
0.3065th %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

MASP1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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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 ↗