Article
Lysosomal protein thermal stability does not correlate with cellular half-life: global observations and a case study of tripeptidyl-peptidase 1.
The Biochemical journal - 14 Feb 2020
Collier Aaron M, Nemtsova Yuliya, Kuber Narendra, Banach-Petrosky Whitney, Modak Anurag, Sleat David E, Nanda Vikas, Lobel Peter
Abstract excerpt
Late-infantile neuronal ceroid lipofuscinosis (LINCL) is a neurodegenerative lysosomal storage disorder caused by mutations in the gene encoding the protease tripeptidyl-peptidase 1 (TPP1). Progression of LINCL can be slowed or halted by enzyme replacement therapy, where recombinant human TPP1 is administered to patients. In this study, we utilized protein engineering techniques to increase the stability of...
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