Mannose Associated Serine Protease 1 (MASP1) Antibody – Rabbit Polyclonal

MASP1: Functions in the lectin pathway of complement, which performs a key role in innate immunity by recognizing pathogens through patterns of sugar moieties and neutralizing them. The lectin pathway is triggered upon binding of mannan-binding lectin (MBL) and ficolins to sugar moieties which leads to activation of the associated proteases MASP1 and MASP2. Functions as an endopeptidase and may activate MASP2 or C2 or directly activate C3 the key component of complement reaction. Isoform 2 may have an inhibitory effect on the activation of the lectin pathway of complement or may cleave IGFBP5. Defects in MASP1 are the cause of 3MC syndrome type 1 (3MC1). 3MC1 is a disorder characterized by facial dysmorphism that includes hypertelorism, blepharophimosis, blepharoptosis and highly arched eyebrows, cleft lip and/or palate, craniosynostosis, learning disability and genital, limb and vesicorenal anomalies. The term 3MC syndrome includes Carnevale, Mingarelli, Malpuech, and Michels syndromes. Belongs to the peptidase S1 family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.4.21.-; Protease; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 16|16 B1

Cellular Component: extracellular region; extracellular space

Molecular Function: calcium ion binding; calcium-dependent protein binding; hydrolase activity; metal ion binding; peptidase activity; protein homodimerization activity; serine-type endopeptidase activity; serine-type peptidase activity

Biological Process: complement activation, lectin pathway; immune system process; innate immune response; proteolysis