CLN1 (I65) polyclonal antibody

Product Specifications

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.

CAS Number

9007-83-4

Structure Composition

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

Product Name Alternative

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

Swiss Prot

P50897

Reactivity

Human, Mouse, Rat

Immunogen

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

Conjugation

Unconjugated

Applications

WB IHC

Dilution

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

Purity

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

Modification

Unmodification

Molecular Weight

~ 34 kDa

Storage Conditions

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

Notes

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

Specificity

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

Applications Notes

Western blot (WB) analysis of CLN1 (I65) pAb at 1:1000 dilution Lane1:A549 whole cell lysate (40ug) Lane2:HepG2 whole cell lysate (40ug) Lane3:The Brain tissue lysate of Mouse (40ug) Lane4:The Spleen tissue lysate of Rat (40ug)