Journal
JOURNAL OF MOLECULAR BIOLOGY
Volume 432, Issue 18, Pages 5043-5051Publisher
ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
DOI: 10.1016/j.jmb.2020.03.026
Keywords
cardiolipin; mitochondria; Barth syndrome; membrane lipids
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Funding
- National Institutes of Health [R01GM115593]
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Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid- lysophospholipid transacylation. The reaction alters the molecular species composition and, as a result, the physical properties of lipids. In vivo, the most important substrate of tafazzin is the mitochondria-specific lipid cardiolipin. Tafazzin mutations cause the human disease Barth syndrome, which presents with cardiomyopathy, skeletal muscle weakness, fatigue, and other symptoms, probably all related to mitochondria! dysfunction. The reason why mitochondria require tafazzin is still not known, but recent evidence suggests that tafazzin may lower the energy cost associated with protein crowding in the inner mitochondrial membrane. (C) 2020 Elsevier Ltd. All rights reserved.
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