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]
Catalog No
L0243
Reactivity
Human, Rat, Mouse
Applications
WB, ELISA
Modification
Cleaved Specific
Source
Polyclonal Rabbit
Dilution
Western Blot: 1/500 - 1/2000. ELISA: 1/20000. Not yet tested in other applications.
Purification
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
The antiserum was produced against synthesized peptide derived from human Dipeptidyl-peptidase 1. AA range:345-394
Specificity
Cleaved-Cathepsin C HC (R394) Polyclonal Antibody detects endogenous levels of fragment of activated Cathepsin C HC protein resulting from cleavage adjacent to R394.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Western blot analysis of lysates from Jurkat cells, treated with etoposide 25uM 1h, using Dipeptidyl-peptidase 1 (heavy chain,Cleaved-Arg394) Antibody. The lane on the right is blocked with the synthesized peptide.
