Palmitoyl-protein thioesterase-1 (PPT1) is a lysosomal hydrolase that removes long-chain fatty acyl groups from modified cysteine residues in proteins.
Mutations in PPT1 have been found to cause the infantile form of neuronal ceroid lipofuscinosis (INCL), and an animal model has been developed.1 The deduced PPT2 protein contains 302 amino acids, including a 27aa leader peptide, a sequence motif characteristic of many thioesterases and lipases, and 5 potential N-linked glycosylation sites.2 PPT2 shares 18% amino acid identity with PPT1.
Northern blot analysis detected a predominant 2.0-kb PPT2 transcript in the human tissues examined, with the highest expression in skeletal muscle; variable amounts of 2.8- and 7.0-kb transcripts were also observed.
Recombinant PPT2, like PPT1, possesses thioesterase activity and localizes to the lysosome.
Since PPT2 could not substitute for PPT1 in correcting the metabolic defect in INCL cells and was unable to remove palmitate groups from palmitoylated proteins that are routinely used as substrates for PPT1it has been postulated that PPT2 possesses a different substrate specificity than PPT1.
Applications:Suitable for use in ELISA, Western Blot, and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:1,000Western Blot: 1:100-1:500Immunohistochemistry: 1:50-1:100Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for at least 12 months.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein G affinity chromatography.
Form：Supplied as a liquid in PBS, 0.09% sodium azide.
Specificity：Recognizes human PPT1. Species Crossreactivity: mouse.
Isotype：IgG
Calc Applications Abbrev：E IHC WB
Calc Crossreactivity：Hu Mo
Immunogen：Synthetic peptide selected from the N-terminal region of human PPT1 (KLH).
