Cathepsin C (CTSC), Polyclonal Antibody

CTS-C; DPPI; CPPI; DPP1; DPP-I; HMS; JP; JPD; PALS; PLS; Dipeptidyl Peptidase I; Dipeptidyl transferase; Cathepsin J

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-CTSC antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC)

15,700 Da

cathepsin C

dipeptidyl peptidase 1

Dipeptidyl peptidase 1

CPPI; DPP-I; DPPI  [Similar Products]

This gene encodes a member of the peptidase C1 family and lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in cells of the immune system. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate heavy and light chains that form a disulfide-linked dimer. A 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. [provided by RefSeq, Nov 2015]

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; Endoplasmic reticulum; Protease

Chromosomal Location of Human Ortholog: 11q14.2

Cellular Component: centrosome; COPII-coated ER to Golgi transport vesicle; endoplasmic reticulum lumen; endoplasmic reticulum-Golgi intermediate compartment membrane; extracellular region; extracellular space; Golgi membrane; intracellular membrane-bound organelle; lysosome; membrane; nucleoplasm

Molecular Function: chaperone binding; chloride ion binding; cysteine-type endopeptidase activity; cysteine-type peptidase activity; identical protein binding; peptidase activator activity involved in apoptotic process; phosphatase binding; protein binding; protein self-association; serine-type endopeptidase activity

Biological Process: aging; apoptosis; COPII vesicle coating; ER to Golgi vesicle-mediated transport; immune response; neutrophil degranulation; proteolysis; proteolysis involved in cellular protein catabolic process; response to organic substance; T cell mediated cytotoxicity

Disease: Haim-munk Syndrome; Papillon-lefevre Syndrome; Periodontitis, Aggressive, 1

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