- Catalog number
- PA2084-iFluor647
- Size / count
- 100 µg
- Dry ice
- No
- Hazardous
- No
- Discontinued
- Yes
- Background
- 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.
- Synonyms
- Neuroendocrine convertase 1;NEC 1;3.4.21.93;Prohormone convertase 1;Proprotein convertase 1;PC1;PCSK1;NEC1;
- Gene Name
- Neuroendocrine convertase 1
- UniProt
- P29120
- Host
- Rabbit
- Reactivity
- Human,Mouse,Rat
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human PC1/3, identical to the related mouse and rat sequences.
- Clonality
- Polyclonal
- Tissue Specificity
- 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. .
- Applications
- IF,IHC,WB
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- 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.
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 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.
- Storage Conditions
- Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 84152 MW
- Observed Molecular Weight
- 84 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Western blot, 0.1-0.5μg/ml, Human, Mouse, Rat, <br>
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human, Mouse, Rat, By Heat<br>
Immunofluorescence, 2μg/ml, Human
- Subcellular Location
- Cytoplasmic vesicle, secretory vesicle. Localized in the secretion granules.
- Sequence Similarities
- Belongs to the peptidase S8 family. Furin subfamily.
- Protein Name
- Neuroendocrine convertase 1