|Immunohistochemistry (Paraffin) (IHC (P))||1:50-1:100|
|Western Blot (WB)||1:1000|
|Tested Species reactivity||Human|
|Host / Isotype||Rabbit / IgG|
|Immunogen||KLH conjugated synthetic peptide between 269-300 amino acids from the C-terminal region of human PPT1|
|Purification||Ammonium sulfate precipitation, Size-exclusion - Dialysis|
|Contains||0.09% sodium azide|
|Storage conditions||-20° C, Avoid Freeze/Thaw Cycles|
This antibody is predicted to react with non-human primate based on sequence homology.
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 27-amino acid 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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: ceroid-palmitoyl-palmitoyl-protein thioesterase 1; Palmitoyl-protein hydrolase 1; Palmitoyl-protein thioesterase 1; PPT-1
Gene Aliases: CLN1; INCL; PPT; PPT1
UniProt ID: (Human) P50897
Entrez Gene ID: (Human) 5538
If an Invitrogen™ antibody doesn’t perform as described on our website or datasheet, we’ll replace the product at no cost to you, or provide you with a credit for a future purchase.*
Get expert recommendations for common problems or connect directly with an on staff expert for technical assistance related to applications, equipment and general product use.