The functions of cardiolipin in cellular metabolism-potential modifiers of the Barth syndrome phenotype.
The phospholipid cardiolipin (CL) plays a role in many cellular functions and signaling pathways both inside and outside of mitochondria. This review focuses on the role of CL in energy metabolism. Many reactions of electron transport and oxidative phosphorylation, the transport of metabolites required for these processes, and the stabilization of electron transport chain supercomplexes require CL. Recent studies indicate that CL is required for the synthesis of iron-sulfur (Fe-S) co-factors, which are essential for numerous metabolic pathways. Activation of carnitine shuttle enzymes that are required for fatty acid metabolism is CL dependent. The presence of substantial amounts of CL in the peroxisomal membrane suggests that CL may be required for peroxisomal functions. Understanding the role of CL in energy metabolism may identify physiological modifiers that exacerbate the loss of CL and underlie the variation in symptoms observed in Barth syndrome, a genetic disorder of CL metabolism.
Department of Biological Sciences, Wayne State University, Detroit, United States.
Vaishnavi
V
Raja
Department of Biological Sciences, Wayne State University, Detroit, United States. Electronic address: mlgreen@sun.science.wayne.edu.
Miriam L
ML
Greenberg
D000818:Animals; D056889:Barth Syndrome / Q000378:metabolism* / Q000473:pathology; D002308:Cardiolipins / Q000737:chemistry / Q000378:metabolism*; D004734:Energy Metabolism; D006706:Homeostasis; D006801:Humans; D008928:Mitochondria / Q000378:metabolism; D010641:Phenotype
D002308:Cardiolipins
Barth syndrome; Bioenergetics; Carnitine transport; Fatty acid utilization; Iron–sulfur biogenesis