Aliases
JP; HMS; JPD; PLS; CPPI; DPP1; DPPI; PALS; DPP-I; PDON1
Antibody Type
Polyclonal Antibody
Uniprot ID
Swiss-Prot#: P53634
NCBI Gene ID: 1075
Gen
Immunogen
Synthetic peptide corresponding to a region derived from internal residues of human cathepsin C
Raised In
Rabbit
Species Reactivity
Human Mouse Rat
Tested Applications
WB IHC Recommended dilution: Western blotting: 1:500-1:2000, Immunohistochemistry: 1:50-1:200
Background / Function
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.
Conjugate
Unconjugated
Storage Buffer
Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Form
liquid
Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.
Purity
Antigen Affinity Purified