Home  >  Products  >  TPP-1 (Tripeptidyl-peptidase 1, Tripeptidyl, TPP-I aminopeptidase, Lysosomal pepstatin-insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein, TPP1, CLN2)
TPP-1 (Tripeptidyl-peptidase 1, Tripeptidyl, TPP-I aminopeptidase, Lysosomal pepstatin-insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein, TPP1, CLN2)

TPP-1 (Tripeptidyl-peptidase 1, Tripeptidyl, TPP-I aminopeptidase, Lysosomal pepstatin-insensitive protease, LPIC, Cell growth-inhibiting gene 1 protein, TPP1, CLN2)

Cat no: T8570-01

Supplier: United States Biological
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TPP1 is a lysosomal serine protease with tripeptidyl-peptidase I activity. It may act as a non-specific lysosomal peptidase which releases an N-terminal tripeptide from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. TPP1 is detected in lysosomes and melanosomes in all tissues but it is highest in the heart and placenta. TPP1 is activated by autocatalytic proteolytic processing upon acidification within lysosomes. Defects in TPP1 cause classical late-infantile neuronal ceroid lipofuscinosis (LINCL) which is also known as ceroid lipofuscinosis 2 (CLN2). It is a fatal childhood neurodegenerative disease characterised by progressive visual and mental deterioration, motor disturbance, epilepsy and behavioral changes. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:4000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Catalogue number: T8570-01
Reactivities: Human
Hosts: Goat
Applications: ELISA, Western Blot
Size: 100ug
Form: Supplied as a liquid in Tris saline, 0.02% sodium azide, pH7.3, 0.5% BSA.
P type: Pab
Isotype: IgG
Purity: Purified by affinity chromatography.
References: 1. Kousi M, Siintola E, Dvorakova L, Vlaskova H, Turnbull J, Topcu M, Yuksel D, Gokben S, Minassian BA, Elleder M, Mole SE, Lehesjoki AE. Mutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis. Brain : a journal of neurology 2009 Mar 132 (Pt 3): 810-9. PMID: 19201763
Additional info: Recognizes human CLN2/TPP1. Sequence Homology: bovine, canine.

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