The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
IHC-P: Use at a concentration of 3 µg/ml. Antigen retrieval is recommended Place sample in 1X citrate buffer (pH 6.0) and microwave at 750W for 20 minutes, cool sample
WB: Use at a concentration of 1 µg/ml. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons.
Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1) [MIM:256730]. A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late-infantile, juvenile, and adult onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD).
Belongs to the palmitoyl-protein thioesterase family.
Sima N et al. Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses. Orphanet J Rare Dis13:54 (2018).
Read more (PubMed: 29631617) »
Shyng C et al. Widespread Expression of a Membrane-Tethered Version of the Soluble Lysosomal Enzyme Palmitoyl Protein Thioesterase-1. JIMD Rep36:85-92 (2017).
Read more (PubMed: 28213849) »