- Catalog number
- PA2130-APC
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Proprotein convertase subtilisin/kexin type 9, also known as PCSK9, is an enzyme that in humans is encoded by the PCSK9 gene. This gene encodes a proprotein convertase belonging to the proteinase K subfamily of the secretory subtilase family. By genomic sequence analysis, PCSK9 was mapped to chromosome 1p32. This gene is a crucial player in the regulation of plasma cholesterol homeostasis. It may prevent the recycling of LDLR from endosomes to the cell surface or it to lysosomes for degradation. PCSK9 can induce ubiquitination of LDLR leading to its subsequent degradation. This gene is involved in the disposal of non-acetylated intermediates of BACE1 in the early secretory pathway.
- Synonyms
- Proprotein convertase subtilisin/kexin type 9;3.4.21.-;Neural apoptosis-regulated convertase 1;NARC-1;Proprotein convertase 9;PC9;Subtilisin/kexin-like protease PC9;PCSK9;NARC1;PSEC0052;
- Gene Name
- Proprotein convertase subtilisin/kexin type 9
- UniProt
- Q8NBP7
- Host
- Rabbit
- Reactivity
- Human
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the C-terminus of human PCSK9.
- Clonality
- Polyclonal
- Tissue Specificity
- Expressed in neuro-epithelioma, colon carcinoma, hepatic and pancreatic cell lines, and in Schwann cells.
- Applications
- 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
- Crucial player in the regulation of plasma cholesterol homeostasis. Binds to low-density lipid receptor family members: low density lipoprotein receptor (LDLR), very low density lipoprotein receptor (VLDLR), apolipoprotein E receptor (LRP1/APOER) and apolipoprotein receptor 2 (LRP8/APOER2), and promotes their degradation in intracellular acidic compartments (PubMed:18039658). Acts via a non-proteolytic mechanism to enhance the degradation of the hepatic LDLR through a clathrin LDLRAP1/ARH-mediated pathway. May prevent the recycling of LDLR from endosomes to the cell surface or it to lysosomes for degradation. Can induce ubiquitination of LDLR leading to its subsequent degradation (PubMed:18799458, PubMed:17461796, PubMed:18197702, PubMed:22074827). Inhibits intracellular degradation of APOB via the autophagosome/lysosome pathway in a LDLR-independent manner. Involved in the disposal of non- acetylated intermediates of BACE1 in the early secretory pathway (PubMed:18660751). Inhibits epithelial Na (+) channel (ENaC)- mediated Na (+) absorption by reducing ENaC surface expression primarily by increasing its proteasomal degradation. Regulates neuronal apoptosis via modulation of LRP8/APOER2 levels and related anti-apoptotic signaling pathways. .
- Components
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.
- References & Citations
- 1. Benjannet, S., Rhainds, D., Hamelin, J., Nassoury, N., Seidah, N. G. The proprotein convertase (PC) PCSK9 is inactivated by furin and/or PC5/6A: functional consequences of natural mutations and post-translational modifications. J. Biol. Chem. 281: 30561-30572, 2006.
2. Cohen, J. C., Boerwinkle, E., Mosley, T. H., Jr., Hobbs, H. H. Sequence variations in PCSK9, low LDL, and protection against coronary heart disease. New Eng. J. Med. 354: 1264-1272, 2006.
3. Kathiresan, S., Melander, O., Anevski, D., Guiducci, C., Burtt, N. P., Roos, C., Hirschhorn, J. N., Berglund, G., Hedblad, B., Groop, L., Altshuler, D. M., Newton-Cheh, C., Orho-Melander, M. Polymorphisms associated with cholesterol and risk of cardiovascular events. New Eng. J. Med. 358: 1240-1249, 2008.
4. Teslovich, T. M., Musunuru, K., Smith, A. V., Edmondson, A. C., Stylianou, I. M., Koseki, M., Pirruccello, J. P., Ripatti, S., Chasman, D. I., Willer, C. J., Johansen, C. T., Fouchier, S. W., and 197 others. Biological, clinical and population relevance of 95 loci for blood lipids. Nature 466: 707-713, 2010.
- 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
- 74286 MW
- Observed Molecular Weight
- 66 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Western blot, 0.1-0.5μg/ml, Human<br>
Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human, By Heat<br>
- Subcellular Location
- Cytoplasm. Secreted. Endosome. Lysosome. Cell surface. Endoplasmic reticulum. Golgi apparatus. Autocatalytic cleavage is required to transport it from the endoplasmic reticulum to the Golgi apparatus and for the secretion of the mature protein. Localizes to the endoplasmic reticulum in the absence of LDLR and colocalizes to the cell surface and to the endosomes/lysosomes in the presence of LDLR. The sorting to the cell surface and endosomes is required in order to fully promote LDLR degradation.
- Sequence Similarities
- Belongs to the peptidase S8 family.
- Protein Name
- Proprotein convertase subtilisin/kexin type 9
