Background: CLN1 (ceroid lipofuscinosis 1), also known as PPT, INCL or PPT1 (palmitoyl-protein thioesterase 1), is a 306 amino acid glycosylated protein that localizes to lysosome and is a member of the palmitoyl-protein thioesterase family. CLN1 functions to remove thioester-linked fatty acyl groups from a variety of substrates, such as as palmitate, from modified cysteine residues in proteins or peptides during lysosomal degradation. Defects in the gene encoding CLN1 are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1 or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, with clinical symptoms including seizures, dementia, visual loss and/or cerebral atrophy.
Description: Rabbit polyclonal to PPT1
Immunogen: KLH conjugated synthetic peptide derived from PPT1
Specificity: ·Reacts with Human, Mouse, Pig, Dog and Rat.
·Isotype: IgG
Application: ·Western blotting: 1/100-500. Predicted Mol wt: 34 kDa;
·Immunohistochemistry (Frozen/paraffin tissue section): 1/50-200;
·Immunocytochemistry/Immunofluorescence: 1/100;
·Immunoprecipitation: 1/50;
·ELISA: 1/500;
·Optimal working dilutions must be determined by the end user.
