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Pro-cathepsin D, Prosaposin, and Progranulin: Lysosomal Networks in Parkinsonism.
Tayebi, Nahid; Lopez, Grisel; Do, Jenny; Sidransky, Ellen.
Afiliación
  • Tayebi N; Medical Genetics Branch, National Human Genetics Research Institute, National Institutes of Health, Bethesda, MD, USA.
  • Lopez G; Medical Genetics Branch, National Human Genetics Research Institute, National Institutes of Health, Bethesda, MD, USA.
  • Do J; Medical Genetics Branch, National Human Genetics Research Institute, National Institutes of Health, Bethesda, MD, USA.
  • Sidransky E; Medical Genetics Branch, National Human Genetics Research Institute, National Institutes of Health, Bethesda, MD, USA. Electronic address: sidranse@mail.nih.gov.
Trends Mol Med ; 26(10): 913-923, 2020 10.
Article en En | MEDLINE | ID: mdl-32948448
Mutations in GBA1, the gene encoding the lysosomal hydrolase glucocerebrosidase (GCase), are a risk factor for parkinsonism. Pursuing the potential mechanisms underlying this risk in aging neurons, we propose a new network uniting three major lysosomal proteins: (i) cathepsin D (CTSD), which plays a major role in α-synuclein (SNCA) degradation and prosaposin (PSAP) cleavage; (ii) PSAP, essential for GCase activation and progranulin (PGRN) transport; and (iii) PGRN, impacting lysosomal biogenesis, PSAP trafficking, and CTSD maturation. We hypothesize that alterations to this network and associated receptors modify lysosomal function and subsequently impact both SNCA degradation and GCase activity. By exploring the interactions between this protein trio and each of their respective transporters and receptors, we may identify secondary risk factors that provide insight into the relationship between these lysosomal proteins, GCase, and SNCA, and reveal novel therapeutic targets.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Catepsina D / Trastornos Parkinsonianos / Saposinas / Precursores Enzimáticos / Progranulinas / Lisosomas Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: Trends Mol Med Asunto de la revista: BIOLOGIA MOLECULAR Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Catepsina D / Trastornos Parkinsonianos / Saposinas / Precursores Enzimáticos / Progranulinas / Lisosomas Tipo de estudio: Prognostic_studies Límite: Animals / Humans Idioma: En Revista: Trends Mol Med Asunto de la revista: BIOLOGIA MOLECULAR Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido