MASP1 : MBL associated serine protease 1


Description

The MASP1 (MBL associated serine protease 1) is a protein-coding gene located on chromosome 3.

The MASP1 gene provides instructions for making proteins involved in the lectin complement pathway. This pathway helps direct cell movement (migration) during development, particularly in neural crest cells, which form tissues like those in the face, skull, endocrine glands, and parts of the nervous system. After birth, the pathway contributes to the immune system. The MASP1 gene produces proteins MASP-1, MASP-3, and MAp44, with variations at one end of their structure. MASP-1 and MASP-3 have different serine protease domains, while MAp44 lacks this domain. Researchers are investigating the specific roles of these proteins in the lectin complement pathway.

MASP1 functions within the lectin pathway of complement, a critical component of innate immunity. This pathway recognizes and neutralizes pathogens by detecting specific sugar patterns on their surfaces. The pathway is activated when mannan-binding lectin (MBL) or ficolins bind to these sugar patterns, leading to the activation of the associated proteases, including MASP1 and MASP2. MASP1 acts as an endopeptidase, potentially activating MASP2 or C2, or directly activating C3, the core component of the complement reaction. A specific isoform, isoform 2, may inhibit the activation of the lectin pathway or cleave IGFBP5. MASP1 also contributes to developmental processes.

MASP1 is also known as 3MC1, CRARF, CRARF1, MAP-1, MAP1, MASP, MASP-3, MASP3, MAp44, PRSS5, RaRF.

Associated Diseases


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