Cathepsin C Active Protein | CTSC active protein

Cathepsin C Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8120308

• Gene Names: CTSC; JP; HMS; JPD; PLS; CPPI; DPP1; DPPI; PALS; DPP-I; PDON1
• Purity: >92% as determined by SDS-PAGE
• Synonyms: Cathepsin C; Cathepsin C Protein; Human; Recombinant (His Tag); Human Cathepsin C/CTSC/DPPI Protein (His Tag); CPPI Protein; DPP-I Protein; DPP1 Protein; DPPI Protein; HMS Protein; JP Protein; JPD Protein; PALS Protein; PDON1 Protein; PLS Protein; cathepsin C; CTSC active protein

• Host: HEK293 Cells
• Purity/Purification: >92% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Met1-Leu463

• Species: Human
• Activity: Measured by its ability to cleave the fluorogenic peptide substrate, Gly-Arg-7-amido-4-methylcoumarin (GRAMC). The specific activity is >200pmoles/min/ug. (Activation description: The proenzyme needs to be activated by Cathepsin L for an activated form)
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Asp 25
• Tag: C-His
• Protein Construction: A DNA sequence encoding the pro form of human cathepsin C (NP_001805.3)(Met 1-Leu 463) was expressed with a polyhistidine tag at the C-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

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Related Product Information for CTSC active protein

Background: Cathepsins are proteases found in many types of cells conserved in all animals, which have a vital role in mammalian cellular turnover such as bone resorption. The lysosomal cysteine protease Cathepsin C (CTSC), also known as dipeptidyl peptidase I (DPPI/DPP1), activates a number of granule-associated serine proteases with pro-inflammatory and immune functions by removal of their inhibitory N-terminal dipeptides. This lysosomal exo-cysteine protease belonging to the peptidase C1 family. Active cathepsin C is found in lysosomes as a 200-kDa multimeric enzyme. Subunits constituting this assembly all arise from the proteolytic cleavage of a single precursor giving rise to three peptides: the propeptide, the alpha- and the beta-chains. It is a central coordinator for activation of many serine proteases in immune/inflammatory cells. Defects in the Cathepsin C have been shown to be a cause of Papillon-Lefevre disease, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis. Cathepsin C plays a key role in the activation of several degradative enzymes linked to tissue destruction in inflammatory diseases. Thus, it is a therapeutic target for the treatment of a number of inflammatory and autoimmune diseases.

References

Santilman V, et al. (2002) Importance of the propeptide in the biosynthetic maturation of rat cathepsin C. Eur J Cell Biol. 81(12): 654-63.Kam CM, et al. (2004) Design and evaluation of inhibitors for dipeptidyl peptidase I (Cathepsin C). Arch Biochem Biophys. 427(2): 123-34.Noack B, et al. (2008) Cathepsin C gene variants in aggressive periodontitis. J Dent Res. 87(10): 958-63.Laine DI, et al. (2010) Inhibitors of cathepsin C (dipeptidyl peptidase I). Expert Opin Ther Pat. 20(4): 497-506.

NCBI and Uniprot Product Information

• NCBI GI #: 189083844
• NCBI GeneID: 1075
• NCBI Accession #: NP_001805.3
• NCBI GenBank Nucleotide #: NM_001814.4
• UniProt Accession #: P53634; Q2HIY8; Q53G93; Q71E75; Q71E76; Q7M4N9; Q7Z3G7; Q7Z5U7; Q8WY99; Q8WYA7; A8K7V2; B5MDD5
• Molecular Weight: 15,700 Da
• NCBI Official Full Name: dipeptidyl peptidase 1 isoform a preproprotein
• NCBI Official Synonym Full Names: cathepsin C
• NCBI Official Symbol: CTSC
• NCBI Official Synonym Symbols: JP; HMS; JPD; PLS; CPPI; DPP1; DPPI; PALS; DPP-I; PDON1
• NCBI Protein Information: dipeptidyl peptidase 1; cathepsin J; dipeptidyl peptidase I; dipeptidyl transferase; dipeptidyl-peptidase I
• UniProt Protein Name: Dipeptidyl peptidase 1
• Protein Family: Cathepsin
• UniProt Gene Name: CTSC
• UniProt Synonym Gene Names: CPPI; DPP-I; DPPI
• UniProt Entry Name: CATC_HUMAN

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; Endoplasmic reticulum; Protease

Chromosomal Location of Human Ortholog: 11q14.2

Cellular Component: Golgi apparatus; extracellular space; membrane; endoplasmic reticulum; lysosome

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

Biological Process: response to organic substance; apoptosis; immune response; proteolysis; T cell mediated cytotoxicity; aging

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