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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. De plus, nous expédions Cathepsin C Kits (33) et Cathepsin C Protéines (19) et beaucoup plus de produits pour cette protéine.
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A compound mutation consisting of a large deletion and a nonsense mutation, which provides a new insight in the mutation type of CTSC gene.
CTSC gene missense mutation is responsible for Papillo- Lefevre syndrome in a Turkish family.
analysis of fluorescent substrates provides a detailed S' specificity study of cathepsin C
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.
During the active remyelinating phase, both CysF knockdown (CysFKD) and microglial-selective CatC overexpression (CatCOE) showed a worsening of the demyelination in Plp (Montrer C3 Anticorps)(4e/-) transgenic mice. Conversely, during the chronic demyelinating phase, CatC knockdown (CatCKD) ameliorated the demyelination. Our results suggest that the balance between CatC and CysF expression controls the demyelination and remyelination process.
Double immunofluorescence analysis showed that CTLA-2alpha was co-localized with cathepsin L (Montrer CTSL1 Anticorps), cathepsin C, and TINAGL1 (Montrer TINAGL1 Anticorps) in placenta.
Data indicate that all-trans retinoic acid (ATRA), which induces miR (Montrer MLXIP Anticorps)-23a expression, decreases cathepsin C (CTSC) expression and granzyme B 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 (Montrer SFTPD Anticorps) 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 (Montrer CXCL13 Anticorps) and Ki-67 (Montrer MKI67 Anticorps) 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 (Montrer IL1B Anticorps) by macrophages, neutrophils, and mast cells in vitro is dependent on caspase-1 (Montrer CASP1 Anticorps) but not on cathepsin C.
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 I
, dipeptidyl transferase
, dipeptidyl-peptidase I
, cathepsin C
, dipeptidyl peptidase 1 isoform a preproprotein
, Dipeptidyl-peptidase 1
, dipeptidyl-peptidase 1
, dipeptidylpeptidase 1