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

Author:

Seminotti BiancaORCID,Brondani MorganaORCID,Ribeiro Rafael TeixeiraORCID,Leipnitz GuilhianORCID,Wajner MoacirORCID

Funder

Conselho Nacional de Desenvolvimento Científico e Tecnológico

Fundação de Amparo à Pesquisa do Estado do Rio Grande do Sul

Fundo de Incentivo à Pesquisa e Eventos / Hospital de Clínicas de Porto Alegre

Financiadora de Estudos e Projetos / Rede Instituto Brasileiro de Neurociências

Instituto Nacional de Ciência e Tecnologia para Excitotoxicidade e Neuroproteção

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior

Publisher

Springer Science and Business Media LLC

Subject

Neuroscience (miscellaneous),Cellular and Molecular Neuroscience,Neurology

Reference86 articles.

1. Goodman SI, Markey SP, Moe PG, Miles BS, Teng CC (1975) Glutaric aciduria; a “new” disorder of amino acid metabolism. Biochem Med 12:12–21. https://doi.org/10.1016/0006-2944(75)90091-5

2. Goodman SI, Kohlhoff JG (1975) Glutaric aciduria: inherited deficiency of glutaryl-CoA dehydrogenase activity. Biochem Med 13:138–140. https://doi.org/10.1016/0006-2944(75)90149-0

3. Larson A, Goodman S (2019) Glutaric acidemia type 1 summary genetic counseling. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A (eds) GeneReviews®. University of Washington, Seattle, pp 1–27

4. Wajner M (2019) Neurological manifestations of organic acidurias. Nat Rev Neurol 15:253–271. https://doi.org/10.1038/s41582-019-0161-9

5. Goodman SI, Frerman FE (2014) Organic acidemias due to defects in lysine oxidation: 2-ketoadipic acidemia and glutaric acidemia. In: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G (eds) The Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill, New York, 2014. http://ommbid.mhmedical.com/content.aspx?bookid=971§ionid=62677296. Accessed 24 January 2022

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