BS2843

antibody from Bioworld Technology, Inc

Targeting: PPT1 CLN1, INCL, PPT

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

BS2843

Provider

Bioworld Technology, Inc

Product name

CLN1 (I65) polyclonal antibody

Antibody type

Polyclonal

Antigen

Synthetic peptide, corresponding to amino acids 50-100 of Human CLN1.

Description

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.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Isotype

IgG

Vial size

100ul

Concentration

1 mg/ml

Storage

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