Mitochondrial translation in health and disease


Autoria(s): Ferreiro-Barros, Claudia C.; Barros, Mário Henrique de
Contribuinte(s)

UNIVERSIDADE DE SÃO PAULO

Data(s)

20/03/2014

20/03/2014

2013

Resumo

Mitochondrial disorders have become the most common cause of inborn errors of metabolism. Impairments in mitochondrial protein synthesis are one of the causes of these diseases, which are clinically and genetically heterogeneous. The mitochondrial translation machinery decodes 13 polypeptides essential for the oxidative phosphorylation process. Mitochondria protein synthesis depends on the integrity of mitochondrial rRNAs and tRNAs genes, and at least one hundred of nuclear encoded products. Diseases caused by mutations in mitochondrial genes as well as in ribosomal proteins, translational factors, RNA modifying enzymes, and all other constituents of the translational machinery have been described in patients with combine respiratory chain deficiency, and are the object of this review.

Fundação de Amparo a Pesquisa de São Paulo (FAPESP - 2010/51924-0)

Fundação de Amparo a Pesquisa de São Paulo (FAPESP - 2011/07366-5)

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Identificador

Open Journal of Endocrine and Metabolic Diseases, Delaware, v.3, p.1-9, 2013

http://www.producao.usp.br/handle/BDPI/44232

10.4236/ojemd.2013.32A001

http://dx.doi.org/10.4236/ojemd.2013.32A001

Idioma(s)

eng

Publicador

SciRes

Delaware

Relação

Open Journal of Endocrine and Metabolic Diseases

Direitos

openAccess

http://creativecommons.org/licenses/by/3.0/br/

Claudia C. Ferreiro-Barros, Mario H. Barros

Palavras-Chave #Mitochondrial Diseases #Respiratory Chain #Metabolismo #Mitocôndrias
Tipo

article

original article

publishedVersion