Over the past decade, it has become clear that lipid homeostasis is central to cellular
metabolism. Lipids are particularly abundant in the central nervous system (CNS) where …

The protonation state of soluble and membrane-associated macromolecules dictates their
charge, conformation, and functional activity. In addition, protons (H+ or their equivalents) …

Found in all organisms, pyruvate dehydrogenase complexes (PDC) are the keystones of
prokaryotic and eukaryotic energy metabolism. In eukaryotic organisms these multi …

Barth syndrome is a rare X-linked genetic disease classically characterized by
cardiomyopathy, skeletal myopathy, growth retardation, neutropenia, and 3 …

The mitochondrial phospholipid cardiolipin (CL) is critical for numerous essential biological
processes, including mitochondrial dynamics and energy metabolism. Mutations in the CL …

The heart is the most energy-consuming organ in the human body. In heart failure, the
homeostasis of energy supply and demand is endangered by an increase in cardiomyocyte …

Cardiolipin (CL) is the signature phospholipid (PL) of mitochondria and plays a pivotal role
in mitochondrial and cellular function. Disruption of the CL remodeling gene tafazzin (TAZ) …

Studies of rare human genetic disorders of mitochondrial phospholipid metabolism have
highlighted the crucial role that membrane phospholipids play in mitochondrial …

Mutation of the gene Tafazzin (TAZ) causes Barth syndrome, an X‐linked disorder
characterized by cardiomyopathy, skeletal muscle weakness, and neutropenia. TAZ is an …

Friedreich ataxia (FRDA) is a serious hereditary neurodegenerative disease, mostly
accompanied with hypertrophic cardiomyopathy, caused by the reduced expression of …