Deficiency of a Retinal Dystrophy Protein, Acyl-CoA Binding Domain-containing 5 (ACBD5), Impairs Peroxisomal ß-Oxidation of Very-long-chain Fatty Acids.

Yagita, Yuichi; Shinohara, Kyoko; Abe, Yuichi; Nakagawa, Keiko; Al-Owain, Mohammed; Alkuraya, Fowzan S; Fujiki, Yukio

Yagita, Yuichi; Shinohara, Kyoko; Abe, Yuichi; Nakagawa, Keiko; Al-Owain, Mohammed; Alkuraya, Fowzan S; Fujiki, Yukio.

Afiliação

Yagita Y; From the Department of Biology and Graduate School of Systems Life Sciences, Kyushu University, 744 Motooka Nishi-ku, Fukuoka 819-0395, Japan.
Shinohara K; From the Department of Biology and Graduate School of Systems Life Sciences, Kyushu University, 744 Motooka Nishi-ku, Fukuoka 819-0395, Japan.
Abe Y; the Medical Institute of Bioregulation, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan, and.
Nakagawa K; From the Department of Biology and Graduate School of Systems Life Sciences, Kyushu University, 744 Motooka Nishi-ku, Fukuoka 819-0395, Japan.
Al-Owain M; the King Faisal Specialist Hospital and Research Center, MBC-03 P. O. Box 3354, Riyadh 11211, Saudi Arabia.
Alkuraya FS; the King Faisal Specialist Hospital and Research Center, MBC-03 P. O. Box 3354, Riyadh 11211, Saudi Arabia.
Fujiki Y; the Medical Institute of Bioregulation, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan, and yfujiki@kyudai.jp.

J Biol Chem ; 292(2): 691-705, 2017 Jan 13.

Article em En | MEDLINE | ID: mdl-27899449

Assuntos

Acil Coenzima A/metabolismo; Proteínas Adaptadoras de Transdução de Sinal/metabolismo; Citosol/metabolismo; Ácidos Graxos/metabolismo; Proteínas de Membrana/metabolismo; Peroxissomos/metabolismo; Acil Coenzima A/genética; Proteínas Adaptadoras de Transdução de Sinal/genética; Animais; Transporte Biológico Ativo/genética; Ácidos Graxos/genética; Células HeLa; Humanos; Proteínas de Membrana/genética; Oxirredução; Peroxissomos/genética; Peroxissomos/patologia; Domínios Proteicos; Coelhos; Distrofias Retinianas/genética; Distrofias Retinianas/metabolismo; Distrofias Retinianas/patologia

Palavras-chave

acyl-CoA-binding protein; fatty acid ß-oxidation; genetic disease; lipid metabolism; neurological disease; peroxisome; retinal degeneration; retinal dystrophy

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Acil Coenzima A / Peroxissomos / Citosol / Proteínas Adaptadoras de Transdução de Sinal / Ácidos Graxos / Proteínas de Membrana Tipo de estudo: Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2017 Tipo de documento: Article

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google