Journal
CELLS
Volume 10, Issue 7, Pages -Publisher
MDPI
DOI: 10.3390/cells10071678
Keywords
spastic paraplegia; lysosomes; autophagy; endosomes
Categories
Funding
- European Union
- Spastic Paraplegia Foundation
- ALS-HSP-France association
- PSL-Biogen program
- French Research Ministry through the EPHE doctoral school [472]
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Hereditary spastic paraplegia (HSP) involves the degeneration of motor neurons and is characterized by clinical and genetic heterogeneity, making it challenging to find effective therapies. Several HSP genes/proteins are linked to the endolysosomal and autophagic pathways, suggesting a functional convergence, which may have implications for understanding the pathological mechanisms of HSP.
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.
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