CLN1 (I65) polyclonal Antibody | BS2843

CLN1 (I65) polyclonal Antibody | BS2843 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse,Rat

Application: WB IHC

Application Range: WB: 1:500~1:1000 IHC: 1:50~1:200

Background: 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.

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

Specificity: CLN1 (I65) polyclonal Antibody detects endogenous levels of CLN1 protein.

Molecular Weight: ~ 34 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Palmitoyl-protein thioesterase 1; PPT-1; 3.1.2.22; Palmitoyl-protein hydrolase 1; PPT1; PPT

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

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: