The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesIHC-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.
FunctionRemoves 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.
Involvement in diseaseDefects 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).
Sequence similaritiesBelongs to the palmitoyl-protein thioesterase family.