Target：CLN1

Fields：Fatty acid elongation;Metabolic pathways;Fatty acid metabolism;Lysosome

Gene Name：PPT1

Protein Name：Palmitoyl-protein thioesterase 1

Human Gene Id：5538

Human Swiss Prot No：P50897

Mouse Gene Id：19063

Mouse Swiss Prot No：O88531

Rat Gene Id：29411

Rat Swiss Prot No：P45479

Immunogen：The antiserum was produced against synthesized peptide derived from human CLN1. AA range:16-65

Specificity：CLN1 Polyclonal Antibody detects endogenous levels of CLN1 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：IHC 1:100 - 1:300. ELISA: 1:10000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：PPT1;PPT;Palmitoyl-protein thioesterase 1;PPT-1;Palmitoyl-protein hydrolase 1

Observed Band(KD)：37kD

Background： The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008],

Function：catalytic activity:Palmitoyl-protein + H(2)O = palmitate + protein.,disease:Defects in PPT1 are a cause of neuronal ceroid lipofuscinosis 4 (CLN4) [MIM:204300]; also known as adult type neuronal ceroid lipofuscinosis (NCL) or Kufs disease.,disease:Defects in PPT1 are the cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1) [MIM:256730]; also called infantile neuronal ceroid lipofuscinosis (INCL). The neuronal ceroid lipofuscinosis are a group of progressive neurodegenerative diseases characterized by the intracellular accumulation of autofluorescent lipopigment storage material in different patterns ultrastructurally. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). There is a core group of four major clinical forms, the infantile, the late-infantile, the juvenile, and the adult forms. The infantile forms are characterized by p

Subcellular Location：Lysosome . Secreted .

Expression： Brain,Cerebellum,Liver,Prostate,Testis,