In vivo evidence of mitochondrial dysfunction and altered redox homeostasis in a genetic mouse model of propionic acidemia: Implications for the pathophysiology of this disorder

Author:

Gallego-Villar L.,Rivera-Barahona A.,Cuevas-Martín C.,Guenzel A.,Pérez B.,Barry M.A.,Murphy M.P.,Logan A.,Gonzalez-Quintana A.,Martín M.A.,Medina S.,Gil-Izquierdo A.,Cuezva J.M.,Richard E.,Desviat L.R.

Funder

Ministerio de Economía y Competitividad

Comunidad de Madrid

Fondo Europeo de Desarrollo Regional-FEDER

Fundación Séneca de la Región de Murcia

Publisher

Elsevier BV

Subject

Physiology (medical),Biochemistry

Reference54 articles.

1. Disorders of propionate and methylmalonate metabolism;Fenton,2001

2. Understanding molecular mechanisms in propionic acidemia and investigated therapeutic strategies;Richard;Expert Opini. Orphan Drugs,2015

3. Natural history of propionic acidemia;Pena;Mol. Genet. Metab.,2012

4. Survey of health status and complications among propionic acidemia patients;Pena;Am. J.Med. Genet. A,2012

5. Propionic acidemia: neonatal versus selective metabolic screening;Grunert;J. Inherit. Metab. Dis.,2011

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