ECHDC1 knockout mice accumulate ethyl-branched lipids and excrete abnormal intermediates of branched-chain fatty acid metabolism.

Dewulf, Joseph P; Paquay, Stéphanie; Marbaix, Etienne; Achouri, Younès; Van Schaftingen, Emile; Bommer, Guido T

Dewulf, Joseph P; Paquay, Stéphanie; Marbaix, Etienne; Achouri, Younès; Van Schaftingen, Emile; Bommer, Guido T.

Afiliação

Dewulf JP; Department of Biochemistry, de Duve Institute, UCLouvain, Brussels, Belgium; Walloon Excellence in Lifesciences and Biotechnology (WELBIO), Brussels, Belgium; Department of Laboratory Medicine, University Hospital St Luc, UCLouvain, Bruxelles, Belgium. Electronic address: joseph.dewulf@uclouvain.be.
Paquay S; Department of Biochemistry, de Duve Institute, UCLouvain, Brussels, Belgium; Walloon Excellence in Lifesciences and Biotechnology (WELBIO), Brussels, Belgium; Department of Neuropediatrics, University Hospital St Luc, UCLouvain, Bruxelles, Belgium.
Marbaix E; Department of Anatomical Pathology, University Hospital St Luc, UCLouvain, Bruxelles, Belgium; Department of Cell Biology, de Duve Institute, UCLouvain, Bruxelles, Belgium.
Achouri Y; Transgenesis Platform, de Duve Institute, UCLouvain, Bruxelles, Belgium.
Van Schaftingen E; Department of Biochemistry, de Duve Institute, UCLouvain, Brussels, Belgium; Walloon Excellence in Lifesciences and Biotechnology (WELBIO), Brussels, Belgium. Electronic address: emile.vanschaftingen@uclouvain.be.
Bommer GT; Department of Biochemistry, de Duve Institute, UCLouvain, Brussels, Belgium; Walloon Excellence in Lifesciences and Biotechnology (WELBIO), Brussels, Belgium. Electronic address: guido.bommer@uclouvain.be.

J Biol Chem ; 297(4): 101083, 2021 10.

Article em En | MEDLINE | ID: mdl-34419447

Assuntos

Acil Coenzima A/metabolismo; Carboxiliases/deficiência; Ácidos Graxos/metabolismo; Acil Coenzima A/genética; Animais; Carboxiliases/metabolismo; Ácidos Graxos/genética; Camundongos; Camundongos Knockout

Palavras-chave

2,2-dimethylmalonic acid; 2,2-dimethylmalonyl-CoA; ECHDC1; acylglycine; acyltaurine; branched-chain FAs; ethyl-branched fatty acids; ethylmalonic acid; ethylmalonyl-CoA; methyl-branched fatty acids; plasmanylcholine

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Acil Coenzima A / Carboxiliases / Ácidos Graxos Limite: Animals Idioma: En Ano de publicação: 2021 Tipo de documento: Article

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