TPP1 Protein, Human, Recombinant (His Tag)

Product Specifications

Background

Tripeptidyl-peptidase 1 (TPP1 / CLN2) is a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. TPP1 / CLN2 may act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Defects in TPP1 / CLN2 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2), a form of neuronal ceroid lipofuscinosis which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. 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.

Overview

A DNA sequence encoding the pro form of human TPP1 (AAH14863.1) (Met 1-Pro 563) was fused with a polyhistidine tag at the C-terminus.

Synonyms

CLN2 Protein, Human; GIG1 Protein, Human; LPIC Protein, Human; SCAR7 Protein, Human; TPP-1 Protein, Human

Gene Name

Tripeptidyl peptidase I

Expression System

Baculovirus-Insect Cells

Tag

C-His

Field of Research

Serine Proteases and Regulators

Endotoxin

< 1.0 EU per μg of the protein as determined by the LAL method.

Purity

> 95 % as determined by SDS-PAGE.

Activity

Measured by the cleavage of AlaAlaPheAMC. The specific activity is > 850 pmoles/min/μg.

Form

Liquid

Buffer

Supplied as sterile 20mM Tris, 500mM NaCl, pH 7.4, 10% gly Please contact us for any concerns or special requirements. Please refer to the specific buffer information in the hard copy of CoA.

Function

Enzyme Proteins

Molecular Weight

The secreted recombinant human TPP1 (pro form) comprises 554 amino acids and has a predicted molecular mass of 60.7 kDa. The apparent molecular mass of rhTPP1 is approximately 60 kDa in SDS-PAGE under reducing conditions.

Storage Conditions

Liquid. It is shipped out with blue ice. Stable for 1 year at -20°C or below from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and opening the cap. For long-term storage, aliquot and store at -20°C or below. Avoid repeated freeze-thaw cycles.