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- W2082553532 endingPage "101" @default.
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- W2082553532 abstract "Many advances have occurred in the field of Barth Syndrome biology in the 26 years since it was first described as an X-linked cardiomyopathy. Barth Syndrome is the first human disease recognized in which the primary causative factor is an alteration in cardiolipin remodeling. Cardiolipin is required for the optimal function of many proteins within the mitochondria, particularly in the respiratory chain and is involved in the mitochondrial-mediated apoptotic process. The appropriate content of cardiolipin appears to be critical for these functions. Cardiolipin is synthesized de novo in mitochondria and is rapidly remodeled to produce CL enriched in linoleic acid. The Barth Syndrome gene TAZ has been identified and expression of the gene yields proteins known as tafazzins. Mutations in TAZ result in a decrease in tetra-linoleoyl species of cardiolipin and an accumulation of monolysocardiolipin within cells from Barth Syndrome patients. Although the protein product of the TAZ gene shows sequence homology to the glycerolipid acyltransferase family of enzymes, its precise biochemical function remains to be elucidated. In this review we highlight some of the recent literature on cardiolipin metabolism and Barth Syndrome." @default.
- W2082553532 created "2016-06-24" @default.
- W2082553532 creator A5013173450 @default.
- W2082553532 creator A5026563745 @default.
- W2082553532 date "2006-03-01" @default.
- W2082553532 modified "2023-10-17" @default.
- W2082553532 title "Cardiolipin metabolism and Barth Syndrome" @default.
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- W2082553532 doi "https://doi.org/10.1016/j.plipres.2005.12.001" @default.
- W2082553532 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16442164" @default.
- W2082553532 hasPublicationYear "2006" @default.
- W2082553532 type Work @default.