l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.

Amino Acid Metabolism, Inborn Errors / metabolism Animals Brain Diseases, Metabolic / metabolism Carnitine / analogs & derivatives Corpus Striatum / metabolism Diet / veterinary Disease Models, Animal Glutaryl-CoA Dehydrogenase / deficiency Glutathione Peroxidase / metabolism Lysine / blood Mice Mice, Knockout Oxidative Stress / drug effects Reactive Oxygen Species / metabolism Superoxide Dismutase / metabolism
Glutaric aciduria type I Glutaryl-CoA dehydrogenase Knockout model Gcdh(−/−) Oxidative stress l-Carnitine

Journal

Biochimica et biophysica acta. Molecular basis of disease
ISSN: 1879-260X
Titre abrégé: Biochim Biophys Acta Mol Basis Dis
Pays: Netherlands
ID NLM: 101731730

Informations de publication

Date de publication:
01 09 2019
Historique:
received: 28 01 2019
revised: 24 05 2019
accepted: 03 06 2019
pubmed: 11 6 2019
medline: 8 5 2020
entrez: 11 6 2019
Statut: ppublish

Résumé

The deficiency of the enzyme glutaryl-CoA dehydrogenase leads to predominant accumulation of glutaric acid (GA) in the organism and is known as glutaric acidemia type I (GA1). Despite the mechanisms of brain damage involved in GA1 are not fully understood, oxidative stress may be involved in this process. Treatment is based on protein/lysine (Lys) restriction and l-carnitine (L-car) supplementation. L-car was recently shown to have an important antioxidant role. A knockout mice model (Gcdh

Identifiants

DOI: 10.1016/j.bbadis.2019.06.007 PMID: 31181292
pubmed: 31181292
pii: S0925-4439(19)30200-5
doi: 10.1016/j.bbadis.2019.06.007
pii:
doi:

Substances chimiques

Reactive Oxygen Species 0
glutarylcarnitine 109006-11-3
Glutathione Peroxidase EC 1.11.1.9
Superoxide Dismutase EC 1.15.1.1
Glutaryl-CoA Dehydrogenase EC 1.3.8.6
Lysine K3Z4F929H6
Carnitine S7UI8SM58A

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

2420-2427

Informations de copyright

Copyright © 2019 Elsevier B.V. All rights reserved.

Auteurs

Gilian Guerreiro (G)

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil. Electronic address: gilianguerreiro@gmail.com.

Alexandre U Amaral (AU)

Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000 Porto Alegre, RS, Brazil.

Rafael Teixeira Ribeiro (RT)

Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000 Porto Alegre, RS, Brazil.

Jéssica Faverzani (J)

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil.

Ana Carolina Groehs (AC)

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil.

Angela Sitta (A)

Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil.

Marion Deon (M)

Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil.

Moacir Wajner (M)

Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000 Porto Alegre, RS, Brazil.

Carmen Regla Vargas (CR)

Faculdade de Farmácia, UFRGS, Av. Ipiranga 2752, 90610-000 Porto Alegre, RS, Brazil; Serviço de Genética Médica, HCPA, UFRGS, Rua Ramiro Barcelos, 2350, 90035-903 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em CB:Bioquímica, UFRGS, Rua Ramiro Barcelos, 2600, 90035 000 Porto Alegre, RS, Brazil; Programa de Pós-Graduação em Ciências Farmacêuticas, UFRGS, Av. Ipiranga, 2752, 90610-000 Porto Alegre, RS, Brazil. Electronic address: crvargas@hcpa.ufrgs.br.

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Classifications MeSH

questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Aminoacidopathies congénitales › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Eucaryotes › Animaux › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Encéphalopathies métaboliques › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Composés chimiques organiques › Amines › Composés de triméthyl-ammonium › Carnitine › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Prosencéphale › Télencéphale › Cerveau › Noyaux gris centraux › Corps strié › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Phénomènes physiologiques › Alimentation et nutrition › Phénomènes physiologiques nutritionnels › Régime alimentaire › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Oxidoreductases acting on CH-CH group donors › Glutaryl-CoA dehydrogenase › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Sélénoprotéines › Glutathione peroxidase › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Acides aminés, peptides et protéines › Acides aminés › Acides aminés essentiels › Lysine › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › Souris transgéniques › Souris knockout › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Phénomènes physiologiques › Stress physiologique › Stress oxydatif › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Produits chimiques inorganiques › Composés de l'oxygène › Espèces réactives de l'oxygène › l-Carnitine prevents oxidative stress in...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Superoxide dismutase › l-Carnitine prevents oxidative stress in...