Anti-PC1/3/PCSK1 Antibody Picoband® Fluoro647 Conjugated

PCSK1 (Proprotein Convertase, Subtilisin/Kexin-Type, 1), also known as PC1 or NEC1, is an enzyme that in humans is encoded by the PCSK1 gene. Proprotein convertase-1 is a neuroendocrine convertase that belongs to a family of subtilisin-like serine endoproteases that process large precursor proteins into mature bioactive products. By in situ hybridization, Seidah et al. (1991) mapped the NEC1 gene to human chromosome 5q15-q21 and mouse chromosome 13. Ohagi et al. (1996) noted that PC1 initiates the sequential processing of proinsulin to insulin by cleaving the proinsulin molecule on the C-terminal side of the dibasic peptide, arg31-arg32, joining the B-chain and C-peptide. By observing the phenotypic features in patients with PC1 mutations, Jackson et al. (2003) concluded that human intestinal absorptive function is dependent on PC1 activity.

Highly expressed on high endothelial venules (HEV) and lamina propia venules found in the small intestine, and to a lesser extent in the colon and spleen. Very low levels of expression found in pancreas and brain. Not expressed in the thymus, prostate, ovaries, testis, heart, placenta, lung, liver, skeletal muscle, kidney or peripheral blood leukocytes. .

Cancer, Cancer Metabolism, Cell Biology, Endocrine Metabolism, Growth Factors/Hormones, Hormone Biosynthesis, Metabolic Signaling Pathway, Metabolism, Pathways and Processes, Proteolysis/Ubiquitin, Proteolytic Enzymes, Signal Transduction

Involved in the processing of hormone and other protein precursors at sites comprised of pairs of basic amino acid residues. Substrates include POMC, renin, enkephalin, dynorphin, somatostatin and insulin.

1. Jackson, R. S., Creemers, J. W. M., Farooqi, I. S., Raffin-Sanson, M.-L., Varro, A., Dockray, G. J., Holst, J. J., Brubaker, P. L., Corvol, P., Polonsky, K. S., Ostrega, D., Becker, K. L., and 10 others. Small-intestinal dysfunction accompanies the complex endocrinopathy of human proprotein convertase 1 deficiency. J. Clin. Invest. 112: 1550-1560, 2003. 2. Ohagi, S., Sakaguchi, H., Sanke, T., Tatsuta, H., Hanabusa, T., Nanjo, K. Human prohormone convertase 3 gene: exon-intron organization and molecular scanning for mutations in Japanese subjects with NIDDM. Diabetes 45: 897-901, 1996. 3. Seidah, N. G., Mattei, M. G., Gaspar, L., Benjannet, S., Mbikay, M., Chretien, M. Chromosomal assignments of the genes for neuroendocrine convertase PC1 (NEC1) to human 5q15-21, neuroendocrine convertase PC2 (NEC2) to human 20p11.1-11.2, and furin (mouse 7[D1-E2] region) . Genomics 11: 103-107, 1991.