Expression of human monolysocardiolipin acyltransferase-1 improves mitochondrial function in Barth syndrome lymphoblasts

The mitochondrial polyglycerophospholipid cardiolipin (CL) is remodeled to obtain specific fatty acyl chains. This is predominantly accomplished by the transacylase enzyme tafazzin (TAZ). Barth syndrome (BTHS) patients with TAZ gene mutations exhibit impaired TAZ activity and loss in mitochondrial r...

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Veröffentlicht in:	The Journal of biological chemistry 2018-05, Vol.293 (20), p.7564-7577
Hauptverfasser:	Mejia, Edgard M., Zegallai, Hana, Bouchard, Eric D., Banerji, Versha, Ravandi, Amir, Hatch, Grant M.
Format:	Artikel
Sprache:	eng
Schlagworte:	Acyltransferases - genetics Acyltransferases - metabolism Barth Syndrome - enzymology Barth Syndrome - pathology Barth Syndrome - prevention & control cardiolipin Cardiolipins - metabolism Case-Control Studies Cells, Cultured Fatty Acids - metabolism genetic disease Humans lipid metabolism Lipids Lymphocytes - enzymology Lysophospholipids - metabolism Mitochondria - metabolism Mitochondria - pathology mitochondrial disease Mutation phospholipid metabolism
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