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.