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.
Applications:Suitable for use in Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.05% sodium azide, pH 7.2.
Specificity：Recognizes endogenous levels of CLN1. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：IHC
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to amino acids 50-100 of Human CLN1.
