TPP1 Proteins, Antibodies, cDNA Clones Research Reagents

TPP1 (Tripeptidyl Peptidase 1) is a protein coding gene located on human chromosome 11p15.4. TPP1 is also known as CLN2, GIG1, LPIC, SCAR7, and TPP-1. The human TPP1 gene encodes a 61248 Da protein containing 563 amino acids. The TPP1 protein is ubiquitously expressed in spleen, appendix and other tissues. Among its related pathways are Lysosome and Unfolded Protein Response (UPR). TPP1 is related to serine-type endopeptidase activity and serine-type peptidase activity. TPP1 is associated with some diseases, including Ceroid Lipofuscinosis, Neuronal, 2 and Spinocerebellar Ataxia, Autosomal Recessive 7.

TPP1 Protein (2)

    TPP1 Antibody (2)

      TPP1 cDNA Clone (28)

      TPP1 Lysate (2)

        TPP1 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.

        TPP1 References

        • Xin H, et al. (2007) TPP1 is a homologue of ciliate TEBP-beta and interacts with POT1 to recruit telomerase. Nature. 445(7127): 559-62.
        • O'Connor MS, et al. (2006) A critical role for TPP1 and TIN2 interaction in high-order telomeric complex assembly. Proc Natl Acad Sci U S A. 103(32): 11874-9.
        • Abreu E, et al. (2010) TIN2-tethered TPP1 recruits human telomerase to telomeres in vivo. Mol Cell Biol. 30(12): 2971-82.

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