The gene coding for the depalmitoylation enzyme, palmitoyl-protein thioesterase (PPT1) is mapped to human chromosome 1p32. Ser115-His289-Asp233 amino acids form the catalytic triad in the active site of the protein. Even though PPT1 is expressed in a variety of tissues, inadequacy of PPT1 protein affect nerve cells only in the cerebral, cerebellar cortex and retina, all other cell types remain comparatively unaffected.
Synonyms: Anti-PPT-1; Anti-Palmitoyl-protein hydrolase 1; Anti-Palmitoyl-protein thioesterase 1
Storage: -20C
Application: Anti-PPT1 antibody produced in rabbit has been used for immunofluorescence and western blotting studies. All Prestige Antibodies(R)Powered by Atlas Antibodies is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: Palmitoyl-protein thioesterase-1 (PPT1) enzyme catalyzes the depalmitoylation reaction involving the removal of long-chain fatty acids from modified cysteine residues in proteins. Mutation of this gene leads to infantile neuronal ceroid lipofuscinosis (INCL), which is an autosomal recessive disorder, characterized by brain atrophy, myoclonic jerk and visual abnormalities.
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