An Experimental Model of Neurodegenerative Disease Based on Porcine Hemagglutinating Encephalomyelitis Virus-Related Lysosomal Abnormalities.
Lysosomal abnormalities
Neurodegenerative diseases
Porcine hemagglutinating encephalomyelitis virus
Progranulin
Trehalose
Journal
Molecular neurobiology
ISSN: 1559-1182
Titre abrégé: Mol Neurobiol
Pays: United States
ID NLM: 8900963
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
19
05
2020
accepted:
27
08
2020
pubmed:
3
9
2020
medline:
2
7
2021
entrez:
3
9
2020
Statut:
ppublish
Résumé
Lysosomes are involved in pathogenesis of a variety of neurodegenerative diseases and play a large role in neurodegenerative disorders caused by virus infection. However, whether virus-infected cells or animals can be used as experimental models of neurodegeneration in humans based on virus-related lysosomal dysfunction remain unclear. Porcine hemagglutinating encephalomyelitis virus displays neurotropism in mice, and neural cells are its targets for viral progression. PHEV infection was confirmed to be a risk factor for neurodegenerative diseases in the present. The findings demonstrated for the first time that PHEV infection can lead to lysosome disorders and showed that the specific mechanism of lysosome dysfunction is related to PGRN expression deficiency and indicated similar pathogenesis compared with human neurodegenerative diseases upon PHEV infection. Trehalose can also increase progranulin expression and rescue abnormalities in lysosomal structure in PHEV-infected cells. In conclusion, these results suggest that PHEV probably serve as a disease model for studying the pathogenic mechanisms and prevention of other degenerative diseases.
Identifiants
pubmed: 32876841
doi: 10.1007/s12035-020-02105-y
pii: 10.1007/s12035-020-02105-y
pmc: PMC7463228
doi:
Substances chimiques
DNA-Binding Proteins
0
Progranulins
0
TDP-43 protein, mouse
0
Trehalose
B8WCK70T7I
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
5299-5306Subventions
Organisme : the National Key Research and Development Program of China
ID : 2016YFD0500102
Organisme : National Natural Science Foundation of China
ID : 31902262
Organisme : National Natural Science Foundation of China
ID : 31872446
Organisme : National Natural Science Foundation of China
ID : 31772704
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