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neuroendocrine (prohormone convertase-1, NEC-1, PC1/PC3, PCSK1)
Function:
- role in the processing of hormone & other protein precursors at sites comprised of pairs of basic amino acid residues
- substrates include POMC, renin, enkephalin, dynorphin, somatostatin & insulin
- release of protein hormones, neuropeptides & renin from their precursors, generally by hydrolysis of -Lys-Arg-|- bonds
Cofactor: Ca+2
Structure: belongs to the peptidase S8 family, Furin subfamily
Compartment:
- cytoplasmic vesicle, secretory vesicle
- localized in the secretion granules
Pathology:
- defects in PCSK1 are associated with obesity due to proprotein convertase 1 deficiency
General
glycoprotein
neuroendocrine convertase; prohormone convertase (NEC)
Properties
SIZE: entity length = 753 aa
MW = 84 kD
COMPARTMENT: cytoplasm
MOTIF: signal sequence {1-27}
active site
SITE: 122-410
MOTIF: aspartate residue {D167}
N-glycosylation site {N173}
histidine residue {H208}
cysteine residue {C225}
MODIFICATION: cysteine residue {C374}
cysteine residue {C317}
MODIFICATION: cysteine residue {C347}
cysteine residue {C347}
MODIFICATION: cysteine residue {C317}
cysteine residue {C374}
MODIFICATION: cysteine residue {C225}
serine residue {S382}
N-glycosylation site {N401}
cysteine residue {C467}
MODIFICATION: cysteine residue {C494}
cysteine residue {C494}
MODIFICATION: cysteine residue {C467}
Amphipathic {739-751}
Database Correlations
OMIM correlations
MORBIDMAP 162150
UniProt P29120
PFAM correlations
Kegg hsa:5122
References
- Barr Cell 66:1 1991
- Jackson et al. Nature Genetics 16:303-6, 1997