The glycine

Glycine N-acyltransferase Glycine conjugation Isovaleric acidemia N-isovalerylglycine

Journal

Computational and structural biotechnology journal
ISSN: 2001-0370
Titre abrégé: Comput Struct Biotechnol J
Pays: Netherlands
ID NLM: 101585369

Informations de publication

Date de publication:
2023
Historique:
received: 15 11 2022
revised: 27 01 2023
accepted: 29 01 2023
entrez: 23 2 2023
pubmed: 24 2 2023
medline: 24 2 2023
Statut: epublish

Résumé

Isovaleric acidemia (IVA), due to isovaleryl-CoA dehydrogenase (IVD) deficiency, results in the accumulation of isovaleryl-CoA, isovaleric acid and secondary metabolites. The increase in these metabolites decreases mitochondrial energy production and increases oxidative stress. This contributes to the neuropathological features of IVA. A general assumption in the literature exists that glycine

Identifiants

pubmed: 36817957
doi: 10.1016/j.csbj.2023.01.041
pii: S2001-0370(23)00043-0
pmc: PMC9932296
doi:

Types de publication

Journal Article

Langues

eng

Pagination

1236-1248

Informations de copyright

© 2023 The Authors.

Déclaration de conflit d'intérêts

The authors declare that there are no conflict of interest.

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Auteurs

Stefan Kühn (S)

Focus Area for Human Metabolomics, North-West University, Private Bag X6001, Potchefstroom 2520, South Africa.

Monray E Williams (ME)

Focus Area for Human Metabolomics, North-West University, Private Bag X6001, Potchefstroom 2520, South Africa.

Marli Dercksen (M)

Focus Area for Human Metabolomics, North-West University, Private Bag X6001, Potchefstroom 2520, South Africa.

Jörn Oliver Sass (JO)

Research Group Inborn Errors of Metabolism, Institute for Functional Gene Analytics, Department of Natural Sciences, Bonn-Rhein-Sieg University of Applied Sciences, von-Liebig-Str. 20, 53359 Rheinbach, Germany.

Rencia van der Sluis (R)

Focus Area for Human Metabolomics, North-West University, Private Bag X6001, Potchefstroom 2520, South Africa.

Classifications MeSH