Product Description

CLN1 (I65) Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS2843-50, BS2843-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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 byintracellμlar accumμlation of autofluorescent liposomal material, with clinicalsymptoms including seizures, dementia, visual loss and/or cerebral atrophy.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IHC

Alternate Names: Palmitoyl-protein thioesterase 1, PPT-1, 3.1.2.22, Palmitoyl-protein hydrolase 1, PPT1, PPT

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

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

Dilution: WB: 1:500~1:1000
IHC: 1:50~1:200

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

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

Molecular Weight: ~ 34 kDa

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

SwissProt: P50897

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Research Use Only