Anti-PPT1 antibody produced in rabbit, purified immunoglobulin, buffered aqueous solution

Not available outside of the UK & Ireland.

Biochem/physiol Actions

Palmitoyl-protein thioesterase 1 (PPT1) functions to remove fatty acid associated with lipid-modified proteins from the cysteine residues. Mutations in the CLN1 or the PPT1 gene is implicated in the cognitive and motor decline disorder in infants called the infantile neuronal ceroid lipofuscinosis. PPT1 is glycosylated and is essential for its functionality.

General description

Palmitoyl-protein thioesterase 1 (PPT1) is a lysosomal enzyme 1) It is mapped to human chromosome 1p34.2 and has eight exons. It is depalmitoylation enzyme with a signal sequence and is targeted to lysosomes through mannose 6-phosphate receptor pathway. It is also localized in axons, synaptic vesicles and synaptosomes of neurons.

Immunogen

PPT1 (NP_000301.1, 1 a.a. ~ 306 a.a) full-length human protein.SequenceMASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG

Physical form

Solution in phosphate buffered saline, pH 7.4