The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 µg/µl.
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Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
Cell growth inhibiting gene 1 protein
Cell growth-inhibiting gene 1 protein
Ceroid lipofuscinosis neuronal 2
Ceroid lipofuscinosis neuronal 2 late infantile
Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease)
Growth inhibiting protein 1
Lysosomal pepstatin insensitive protease
Lysosomal pepstatin-insensitive protease
Tripeptidyl peptidase I
Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus.
Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.
Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2) [MIM:204500]. A form of neuronal ceroid lipofuscinosis. 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 CLN2 consists of curvilinear profiles.
Belongs to the peptidase S53 family.
Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity.
Lysosome. Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.