Enzyme replacement therapy with recombinant pro-CTSD (cathepsin D) corrects defective proteolysis and autophagy in neuronal ceroid lipofuscinosis

CTSD (cathepsin D) is one of the major lysosomal proteases indispensable for the maintenance of cellular proteostasis by turning over substrates of endocytosis, phagocytosis and autophagy. Consequently, CTSD deficiency leads to a strong impairment of the lysosomal-autophagy machinery. In mice and hu...

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Detalhes bibliográficos
Publicado no:Autophagy
Main Authors: Marques, André R. A., Di Spiezio, Alessandro, Thießen, Niklas, Schmidt, Lina, Grötzinger, Joachim, Lüllmann-Rauch, Renate, Damme, Markus, Storck, Steffen E., Pietrzik, Claus U., Fogh, Jens, Bär, Julia, Mikhaylova, Marina, Glatzel, Markus, Bassal, Mahmoud, Bartsch, Udo, Saftig, Paul
Formato: Artigo
Idioma:Inglês
Publicado em: Taylor & Francis 2019
Assuntos:
Research Paper
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7158922/
https://ncbi.nlm.nih.gov/pubmed/31282275
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1080/15548627.2019.1637200
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