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Gene Family: Proprotein convertase subtilisin/kexin family (PCSK)

Also known as : "secretory proprotein convertases", "PCs", "Proprotein convertases", "subtilisin/kexin-like proprotein convertases"

Proprotein convertases: Proprotein convertases are a family of proteins that activate other proteins. Many proteins are inactive when they are first synthesized, because they contain chains of amino acids that block their activity. Proprotein convertases remove those chains and activate the protein. The prototypical proprotein convertase is furin. Proprotein convertases have medical significance, because they are involved in many important biological processes, such as cholesterol synthesis. Compounds called proprotein convertase inhibitors can block their action, and block the target proteins from becoming active. Many proprotein convertases, especially furin and PACE4, are involved in pathological processes such as viral infection, inflammation, hypercholesterolemia, and cancer, and have been postulated as therapeutic targets for some of these diseases. [Source: Wikipedia]

Genes contained within the family: 9

Approved Symbol Approved Name Previous Symbols Synonyms Chromosome
PCSK1 proprotein convertase subtilisin/kexin type 1 NEC1 PC1, PC3, SPC3 5q15
PCSK2 proprotein convertase subtilisin/kexin type 2 NEC2 PC2, SPC2 20p12.1
FURIN furin, paired basic amino acid cleaving enzyme FUR, PACE, PCSK3 SPC1 15q26.1
PCSK4 proprotein convertase subtilisin/kexin type 4 PC4, SPC5, DKFZp434B217, MGC34749 19p13.3
PCSK5 proprotein convertase subtilisin/kexin type 5 PC5, PC6, SPC6 9q21.13
PCSK6 proprotein convertase subtilisin/kexin type 6 PACE4 SPC4 15q26
PCSK7 proprotein convertase subtilisin/kexin type 7 PC7, PC8, LPC, SPC7 11q23.3
MBTPS1 membrane bound transcription factor peptidase, site 1 PCSK8, SKI-1, KIAA0091, S1P 16q23.3-q24.1
PCSK9 proprotein convertase subtilisin/kexin type 9 HCHOLA3 NARC-1, FH3 1p32.3