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The protein encoded by CTSC, 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. Additionally we are shipping Cathepsin C Kits (31) and Cathepsin C Proteins (19) and many more products for this protein.
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Mutation screening of the CTSC gene from the two patients revealed the presence of the same homozygous nonsense mutation in Papillon-Lefevre syndrome and Haim-Munk syndromes. Phenotypic variants of the same rare disease caused by mutations of the cathepsin C (CTSC) gene.
Neutrophilic Cathepsin C Is Maturated by a Multistep Proteolytic Process and Secreted by Activated Cells during Inflammatory Lung Diseases.
CTSC was associated with albuminuria in type 2 diabetes patients.
Homozygous mutation 901G>A in exon 7 of CTSC gene is associated with Papillon-Lefevre syndrome.
Results identify a missense mutation in CTSC gene that segregate within a family with Papillon-Lefevre syndrome.
Papillon-Lefevre syndrome cause by homozygous nonsense mutation of cathepsin C gene.
CatC has a role in the selective tuning of innate and adaptive immune responses, relevant to a chronic immune disease, such as atherosclerosis.
cathepsin C in GCF (show GUCY2F Antibodies) does not seem to have an effect on the pathogenesis of periodontal diseases.
The present account of the 148.621 kb homozygous deletion on chromosome 11 is the first report of a mutational mechanism encompassing the whole CTSC gene.
CTSC mutations in 5 Iranian families with Papillon-Lefevre syndrome (PLS)analyzed; modeled the protein for mutations found in 2 of them; presence of this mutation provides evidence for founder CTSC mutations in PLS; this P35delL mutation leads to loss of a leucine residue; results indicate the phenotypes in these 2 patients likely due to CTSC mutations
Double immunofluorescence analysis showed that CTLA-2alpha was co-localized with cathepsin L (show CTSL1 Antibodies), cathepsin C, and TINAGL1 (show TINAGL1 Antibodies) in placenta.
Data indicate that all-trans retinoic acid (ATRA), which induces miR (show MLXIP Antibodies)-23a expression, decreases cathepsin C (CTSC) expression and granzyme B (show Gzmb Antibodies) activity leading to impaired NK cell cytotoxicity.
These results establish that DPPI is a major determinant of survival following Klebsiella pneumoniae lung infection and suggest that the survival disadvantage in DPPI(+/+) mice is in part due to processing of surfactant protein D (show SFTPD Antibodies) by DPPI.
found no significant role for CtsC during mammary carcinogenesis but revealed squamous carcinogenesis to be functionally dependent on CtsC
Late stage cathepsin C, CXCL13 (show CXCL13 Antibodies) and Ki-67 (show MKI67 Antibodies) overexpression correlate with regional neuropathology in a bovine spongiform encephalopathy transgenic murine model.
Proinflammatory cytokines induce expression and release of cathepsin C in microglial cells during neuroinflammatory progression.
The production of IL-1beta (show IL1B Antibodies) by macrophages, neutrophils, and mast cells in vitro is dependent on caspase-1 (show CASP1 Antibodies) but not on cathepsin C.
NADPH oxidase (show NOX1 Antibodies) in antimicrobial host defense against A. fumigatus and B. cepacia, whereas the proteases neutrophil elastase (show ELANE Antibodies), cathepsin G (show CTSG Antibodies), and lysosomal cysteine protease cathepsin C/ dipeptidyl peptidase I are dispensable
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.
, dipeptidyl peptidase 1
, dipeptidyl peptidase 1 isoform a preproprotein
, cathepsin J
, Dipeptidyl-peptidase 1
, dipeptidyl peptidase I
, dipeptidyl transferase
, dipeptidyl-peptidase I
, dipeptidyl-peptidase 1
, dipeptidylpeptidase 1
, Cat P
, cathepsin L-related protein
, cathepsin P
, gene/Cathepsin L-related protein