Molecular weight (DA)
37kD
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.
Dilution rate
IHC: 1/100 - 1/300. ELISA: 1/10000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Palmitoyl-protein thioesterase 1
Other name
PPT1; PPT; Palmitoyl-protein thioesterase 1; PPT-1; Palmitoyl-protein hydrolase 1
Fields
>>Fatty acid elongation;>>Metabolic pathways;>>Fatty acid metabolism;>>Lysosome
Human protein sequence Database
P50897
Mouse protein sequence database
O88531
Rat protein sequence database
P45479
Cellular localization
Lysosome . Secreted .
Tissue expression
Brain,Cerebellum,Liver,Prostate,Testis,
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