Current Knowledge of Endolysosomal and Autophagy Defects in Hereditary Spastic Paraplegia
| Autor: | Liriopé Toupenet Marchesi, G. Stevanin, Marion Leblanc |
|---|---|
| Přispěvatelé: | Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL) |
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
0301 basic medicine
Nervous system autophagy spastic paraplegia Endosome Hereditary spastic paraplegia QH301-705.5 Kinesins Nerve Tissue Proteins Review Degeneration (medical) Biology Genetic Heterogeneity 03 medical and health sciences 0302 clinical medicine lysosomes GTP-Binding Proteins Valosin Containing Protein medicine Humans Biology (General) Pathological Motor Neurons Spastic Paraplegia Hereditary Genetic heterogeneity Autophagy Membrane Proteins Proteins General Medicine medicine.disease 030104 developmental biology medicine.anatomical_structure Gene Expression Regulation endosomes [SDE]Environmental Sciences Neuroscience 030217 neurology & neurosurgery Signal Transduction trans-Golgi Network |
| Zdroj: | Cells Cells, MDPI, 2021, 10 (7), pp.1678. ⟨10.3390/cells10071678⟩ Cells, Vol 10, Iss 1678, p 1678 (2021) |
| ISSN: | 2073-4409 |
| DOI: | 10.3390/cells10071678⟩ |
| Popis: | International audience; Hereditary spastic paraplegia (HSP) refers to a group of neurological disorders involving the degeneration of motor neurons. Due to their clinical and genetic heterogeneity, finding common effective therapeutics is difficult. Therefore, a better understanding of the common pathological mechanisms is necessary. The role of several HSP genes/proteins is linked to the endolysosomal and autophagic pathways, suggesting a functional convergence. Furthermore, impairment of these pathways is particularly interesting since it has been linked to other neurodegenerative diseases, which would suggest that the nervous system is particularly sensitive to the disruption of the endolysosomal and autophagic systems. In this review, we will summarize the involvement of HSP proteins in the endolysosomal and autophagic pathways in order to clarify their functioning and decipher some of the pathological mechanisms leading to HSP.. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|