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CLN1 (I65) Peptide

Cat no: BS2843P

CLN1 (I65) Peptide

CLN1 (ceroid lipofuscinosis 1), also known as PPT, INCL or PPT1 (palmitoylprotein thioesterase 1), is a 306 amino acid glycosylated protein that localizesto lysosome and is a member of the palmitoyl-protein thioesterase family.CLN1 functions to remove thioester-linked fatty acyl groups from a variety ofsubstrates, such as as palmitate, from modified cysteine residues in proteinsorpeptides during lysosomal degradation. Defects in the gene encoding CLN1are a causeof infantile neuronal ceroid lipofuscinosis 1 (CLN1 or INCL) andneuronal ceroid lipofuscinosis 4 (CLN4). Neuronal ceroid lipofuscinoses areprogressive neurodegenerative, lysosomal storage diseases characterized byintracellular accumulation of autofluorescent liposomal material, with clinicalsymptoms including seizures, dementia, visual loss and/or cerebral atrophy.

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SPECIFICATIONS

Catalog Number

BS2843P

Applications

BNI

Form

1 mg/ml in DI water.

Purity

Synthetic peptide CLN1 (I65). (Note: the amino acid sequence is proprietary). The purity is > 98%.

Swiss Prot

P50897

Storage Temp

Store at 4 degree C short term. Aliquot and store at -20 degree C long term. Avoid freeze-thaw cycles.

Additional Info

This peptide can be used with studies using BS2843 CLN1 (I65) pAb.

Alternative Names

Palmitoyl-protein thioesterase 1; PPT-1; 3.1.2.22; Palmitoyl-protein hydrolase 1; PPT1; PPT

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