Properdin D011414

Chemicals and Drugs [D] » Amino Acids, Peptides, and Proteins [D12] » Proteins » Blood Proteins » Serum Globulins » Beta-Globulins » Properdin

Chemicals and Drugs [D] » Amino Acids, Peptides, and Proteins [D12] » Proteins » Globulins » Serum Globulins » Beta-Globulins » Properdin

Description

A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.   MeSH

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