Western Blot (WB)
(Western blot analysis of Cathepsin C HC expression in HEK293T (A), Raw264.7 (B), PC12 (C) whole cell lysates.)
Rabbit Cathepsin C HC Polyclonal Antibody | anti-CTSC antibody
Anti-Cathepsin C HC Antibody
Western Blot (WB)
(Western blot analysis of Cathepsin C HC expression in HEK293T (A), Raw264.7 (B), PC12 (C) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of Cathepsin C HC expression in HEK293T (A), Raw264.7 (B), PC12 (C) whole cell lysates.)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene, a member of the peptidase C1 family, is a lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in immune/inflammatory cells. It is composed of a dimer of disulfide-linked heavy and light chains, both produced from a single protein precursor, and a residual portion of the propeptide acts as an intramolecular chaperone for the folding and stabilization of the mature enzyme. This enzyme requires chloride ions for activity and can degrade glucagon. Defects in the encoded protein have been shown to be a cause of Papillon-Lefevre syndrome, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
CTSC: Thiol protease. Has dipeptidylpeptidase activity. Active against a broad range of dipeptide substrates composed of both polar and hydrophobic amino acids. Proline cannot occupy the P1 position and arginine cannot occupy the P2 position of the substrate. Can act as both an exopeptidase and endopeptidase. Activates serine proteases such as elastase, cathepsin G and granzymes A and B. Can also activate neuraminidase and factor XIII. Defects in CTSC are a cause of Papillon-Lefevre syndrome (PLS); also known as keratosis palmoplantaris with periodontopathia. PLS is an autosomal recessive disorder characterized by palmoplantar keratosis and severe periodontitis affecting deciduous and permanent dentitions and resulting in premature tooth loss. The palmoplantar keratotic phenotype vary from mild psoriasiform scaly skin to overt hyperkeratosis. Keratosis also affects other sites such as elbows and knees. Defects in CTSC are a cause of Haim-Munk syndrome (HMS); also known as keratosis palmoplantaris with periodontopathia and onychogryposis or Cochin Jewish disorder. HMS is an autosomal recessive disorder characterized by palmoplantar keratosis, onychogryphosis and periodontitis. Additional features are pes planus, arachnodactyly, and acroosteolysis. Defects in CTSC are a cause of aggressive periodontititis type 1 (AP1); also known as juvenile periodontitis (JPD) and prepubertal periodontitis (PPP). AP1 is characterized by severe and protracted gingival infections, leading to tooth loss. AP1 inheritance is autosomal dominant. Belongs to the peptidase C1 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.4.14.1; Protease; Endoplasmic reticulum
Chromosomal Location of Human Ortholog: 11q14.2
Cellular Component: Golgi apparatus; extracellular space; membrane; lysosome; endoplasmic reticulum
Molecular Function: identical protein binding; protein binding; protein self-association; serine-type endopeptidase activity; chaperone binding; apoptotic protease activator activity; chloride ion binding; phosphatase binding; cysteine-type peptidase activity
Biological Process: response to organic substance; apoptosis; immune response; proteolysis; aging; T cell mediated cytotoxicity
Disease: Periodontitis, Aggressive, 1; Papillon-lefevre Syndrome; Haim-munk Syndrome
Research Articles on CTSC
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Product Notes
The CTSC ctsc (Catalog #AAA8218852) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Cathepsin C HC Antibody reacts with Human, Mouse, Rat, Bovine, Dog and may cross-react with other species as described in the data sheet. AAA Biotech's Cathepsin C HC can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB (1/500 - 1/1000). Researchers should empirically determine the suitability of the CTSC ctsc for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Cathepsin C HC, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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