Knock-out mouse models of proprotein convertases: unique functions or redundancy?
The members of the proprotein convertase family play a central role in the processing and/or activation of various protein precursors involved in many physiological processes and various pathologies. The proteolysis of these precursors that occur at basic residues within the general motif (K/R)-(X)-(K/R) is mediated by the proprotein convertases PC1/3, PC2, Furin, PACE4, PC4, PC5 and PC7, whereas the proteolysis of precursors within hydrophobic residues performed by the convertase S1P/SKI-1 and the convertase NARC-1/PCSK9 seems to prefer cleavages at the motif LVFAQSIP. Here we provide a comprehensive overview of their remarkable complex roles as revealed by disruption of their genes individually using generalized or conditional approaches.
Creemers
John W M
JW
Laboratory of Biochemical Neuroendocrinology, Center for Human Genetics K.U. Leuven, Belgium.
Khatib
Abdel-Majid
AM
D000818:Animals; D020134:Catalytic Domain; D018360:Crystallography, X-Ray; D045683:Furin / Q000737:chemistry / Q000172:deficiency / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D010641:Phenotype; D045706:Proprotein Convertase 2 / Q000737:chemistry / Q000172:deficiency / Q000378:metabolism; D045702:Proprotein Convertase 5 / Q000172:deficiency / Q000378:metabolism; D043484:Proprotein Convertases / Q000737:chemistry / Q000172:deficiency* / Q000378:metabolism; D013381:Subtilisins / Q000172:deficiency / Q000378:metabolism
C513086:Pcsk6 protein, mouse; D045702:Proprotein Convertase 5; D043484:Proprotein Convertases; D013381:Subtilisins; C107583:proprotein convertase PC7; D045683:Furin; D045706:Proprotein Convertase 2