. Author manuscript; available in PMC: 2007 Sep 4.

Published in final edited form as: Nat Rev Mol Cell Biol. 2002 Oct;3(10):753–766. doi: 10.1038/nrm934

Table 1.

The proprotein convertase family

Proprotein convertase	Size (amino acids)	Tissue distribution	Subcellular localization	Knockout phenotype
Furin	794	Broad	TGN/endosomal	Embryonic lethality (day 10.5), impaired axial rotation
PC7	785	Broad	TGN/endosomal	Viable; misexpression causes thymic defects
PC5/6A^*	915	Broad	Secretory granules	ND
PC5/6B^*	1877	Broad	TGN/endosomal	ND
PACE4	963	Broad	TGN/endosomal	Embryonic lethality (day 15.5), craniofacial and CNS defects
PC1/3	753	Neuroendocrine system	Secretory granules	Mouse: severe growth defects, defective GHRH and POMC processing, hyperproinsulinaemia
				^‡Humans: severe early obesity, adrenocortical insufficiency, hyperproinsulinaemia
PC2	638	Neuroendocrine system	Secretory granules	Hypoglycaemia, proinsulinaemia, glucagon deficiency, defects in opioid peptide processing
PC4	655	Testicular and ovarian germ cells	ND	Males: infertility
				Females: mild infertility

Included are the phenotypes of the knockout mice for furin⁹⁵, PACE4 (REF. ¹⁶⁴), PC2 (REF. ¹⁶⁵), PC4 (REF. ¹⁶⁶), PC7 (REF. ¹⁶⁷ and N. Seidah, personal communication) and PC1/3 (REF. ¹⁶⁸).

PC5/6 is expressed as either the A or B isoform.

^‡

The phenotype of a patient lacking PC1/3 (REF. ¹⁶⁹). See REFS ¹⁵⁰,¹⁵¹ and ¹⁷⁰ for more comprehensive reviews on the PC family. CNS, central nervous system; GHRH, growth hormone-releasing hormone; ND, not determined; PC, proprotein convertase; POMC, proopiomelanocortin; TGN, trans-Golgi network.