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Article

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

The Journal of biological chemistry - 18 May 2018

Mejia Edgard M, Zegallai Hana, Bouchard Eric D, Banerji Versha, Ravandi Amir, Hatch Grant M

Abstract excerpt

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 respiratory function. Previous studies identified monolysocardiolipin acyltransferase-1 (MLCL AT-1) as a...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 29563154
DOI: 10.1074/jbc.RA117.001024
PMCID: PMC5961033

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