Mouse Anti-Human CLN-2
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|Centrifuge vial prior to opening. Reconstitute the antibody with 500 µl sterile PBS and the final concentration is 200 µg/ml.
|Stability and Storage
|Lyophilized samples are stable for 2 years from date of receipt when stored at -70°C. Reconstituted antibody can be aliquoted and stored frozen at < -20°C for at least six months without detectable loss of activity.
|This antibody was produced from a hybridoma (mouse myeloma fused with spleen cells from a mouse) immunized with Human CLN-2 recombinant protein (also called Tripeptidyl-peptidase I)
|recombinant human CLN-2
|TPP1; CLN2; GIG1; LPIC; TPP-1
|Tripeptidyl-peptidase I (TPP1), also known as CLN2, is a lysosomal serine protease detected in all tissues examined, with highest levels shown in heart and placenta. It is an aminopeptidase that cleaves tripeptides from the free N-terminus of polypeptides and may also have endopeptidase activity. Defects in TPP1 are the cause of classical late-infantile neuronal ceroid lipofusionosis, also known as ceroid lipofusionosis neuronal 2 (CLN2), a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy, and behavioral changes.
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