Mitochondrial ABC proteins in health and disease

作者：Zutz Ariane; Gompf Simone; Schaegger Hermann; Tampe Robert^*

来源：Biochimica et Biophysica Acta-Bioenergetics, 2009, 1787(6): 681-690.

DOI：10.1016/j.bbabio.2009.02.009

• Heme biosynthesis
• Intracellular transport
• Iron homeostasis
• Iron-sulfur cluster assembly
• Mitochondrial targeting
• Oxidative stress

ABC transporters represent one of the largest families of membrane proteins that are found in all three phyla of life. Mitochondria comprise up to four ABC systems, ABCB7/ATM1, ABCB10/MDL1, ABCB8 and ABCB6. These half-transporters, which assemble into homodimeric complexes, are involved in a number of key cellular processes, e.g. biogenesis of cytosolic iron-sulfur clusters, heme biosynthesis, iron homeostasis, multidrug resistance, and protection against oxidative stress. Here, we summarize recent advances and emerging themes in our understanding of how these ABC systems in the inner and outer mitochondrial membrane fulfill their functions in important (patho) physiological processes, including neurodegenerative and hematological disorders.

• 出版日期2009-6