Disturbance of Mitochondrial Dynamics, Endoplasmic Reticulum-Mitochondria Crosstalk, Redox Homeostasis, and Inflammatory Response in the Brain of Glutaryl-CoA Dehydrogenase-Deficient Mice: Neuroprotective Effects of Bezafibrate.
Amino Acid Metabolism, Inborn Errors
/ metabolism
Animals
Bezafibrate
/ pharmacology
Brain
/ metabolism
Brain Diseases, Metabolic
Endoplasmic Reticulum
/ metabolism
Glutaryl-CoA Dehydrogenase
/ deficiency
Homeostasis
Humans
Mice
Mice, Knockout
Mitochondria
/ metabolism
Mitochondrial Dynamics
Neuroprotective Agents
/ metabolism
Oxidation-Reduction
Bezafibrate
Endoplasmic reticulum stress
Glutaric acidemia type 1
Inflammation
Mitochondrial dynamics
Oxidative stress
Journal
Molecular neurobiology
ISSN: 1559-1182
Titre abrégé: Mol Neurobiol
Pays: United States
ID NLM: 8900963
Informations de publication
Date de publication:
Aug 2022
Aug 2022
Historique:
received:
28
01
2022
accepted:
17
05
2022
pubmed:
1
6
2022
medline:
12
8
2022
entrez:
31
5
2022
Statut:
ppublish
Résumé
Patients with glutaric aciduria type 1 (GA1), a neurometabolic disorder caused by deficiency of glutaryl-CoA dehydrogenase (GCDH) activity, commonly manifest acute encephalopathy associated with severe striatum degeneration and progressive cortical and striatal injury whose pathogenesis is still poorly known. We evaluated redox homeostasis, inflammatory response, mitochondrial biogenesis and dynamics, endoplasmic reticulum (ER)-mitochondria crosstalk, and ER stress in the brain of GCDH-deficient (Gcdh
Identifiants
pubmed: 35639256
doi: 10.1007/s12035-022-02887-3
pii: 10.1007/s12035-022-02887-3
doi:
Substances chimiques
Neuroprotective Agents
0
Glutaryl-CoA Dehydrogenase
EC 1.3.8.6
Bezafibrate
Y9449Q51XH
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
4839-4853Subventions
Organisme : Conselho Nacional de Desenvolvimento Científico e Tecnológico
ID : 425914/2016-0
Organisme : Conselho Nacional de Desenvolvimento Científico e Tecnológico
ID : 427051/2018-5
Organisme : Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul
ID : 16/0465-0
Organisme : Fundo de Incentivo à Pesquisa e Eventos / Hospital de Clínicas de Porto Alegre
ID : 180538
Organisme : Financiadora de Estudos e Projetos / Rede Instituto Brasileiro de Neurociências
ID : 01.06.0842-00
Organisme : Instituto Nacional de Ciência e Tecnologia para Excitotoxicidade e Neuroproteção
ID : 465671/2014-4
Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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