Product Description
CLN1 (I65) polyclonal Antibody | BS2843 | Bioworld
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: