Lack of p62 Impairs Glycogen Aggregation and Exacerbates Pathology in a Mouse Model of Myoclonic Epilepsy of Lafora.
Epilepsy
Glycogen
Lafora bodies
Lafora disease
Malin
Neuroinflammation
p62
Journal
Molecular neurobiology
ISSN: 1559-1182
Titre abrégé: Mol Neurobiol
Pays: United States
ID NLM: 8900963
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
received:
31
08
2021
accepted:
04
12
2021
pubmed:
29
12
2021
medline:
8
4
2022
entrez:
28
12
2021
Statut:
ppublish
Résumé
Lafora disease (LD) is a fatal childhood-onset dementia characterized by the extensive accumulation of glycogen aggregates-the so-called Lafora Bodies (LBs)-in several organs. The accumulation of LBs in the brain underlies the neurological phenotype of the disease. LBs are composed of abnormal glycogen and various associated proteins, including p62, an autophagy adaptor that participates in the aggregation and clearance of misfolded proteins. To study the role of p62 in the formation of LBs and its participation in the pathology of LD, we generated a mouse model of the disease (malin
Identifiants
pubmed: 34962634
doi: 10.1007/s12035-021-02682-6
pii: 10.1007/s12035-021-02682-6
pmc: PMC8857170
doi:
Substances chimiques
Sequestosome-1 Protein
0
Sqstm1 protein, mouse
0
Glycogen
9005-79-2
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1214-1229Subventions
Organisme : Horizon 2020
ID : Marie Skłodowska-Curie grant agreement No. 75451
Organisme : Instituto de Salud Carlos III
ID : CIBERDEM
Organisme : NINDS NIH HHS
ID : P01 NS097197
Pays : United States
Organisme : Departament d'Innovació, Universitats i Empresa, Generalitat de Catalunya
ID : SGR2017-648
Organisme : Ministerio de Ciencia, Innovación y Universidades
ID : BFU2017-84345-P
Organisme : Agencia Estatal de Investigación
ID : RT2018-099773-B-100
Organisme : NINDS NIH HHS
ID : P01NS097197
Pays : United States
Organisme : Ministerio de Ciencia, Innovación y Universidades
ID : PID202-118699GB-I00
Informations de copyright
© 2021. The Author(s).
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