Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers

Author:

Seminotti Bianca12,Leipnitz Guilhian12,Karunanidhi Anuradha1,Kochersperger Catherine1,Roginskaya Vera Y3,Basu Shrabani1,Wang Yudong1,Wipf Peter4,Van Houten Bennett3,Mohsen Al-Walid15,Vockley Jerry15

Affiliation:

1. Division Medical Genetics, Department of Pediatrics, University of Pittsburgh, Pittsburgh, PA, USA

2. Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

3. Department of Pharmacology and Chemical Biology, University of Pittsburgh, Pittsburgh, PA, USA

4. Department of Chemistry, University of Pittsburgh, Pittsburgh, PA, USA

5. Department of Human Genetics, Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA, USA

Funder

National Institutes of Health

Publisher

Oxford University Press (OUP)

Subject

Genetics (clinical),Genetics,Molecular Biology,General Medicine

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