Mitochondria in health and disease

Published on May 31, 2020   35 min
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My name is Eamonn Maher, I'm Professor of Medical Genetics and Genomic Medicine and Honorary Consultant in Clinical Genetics in the Department of Medical Genetics at the University of Cambridge and Cambridge University Hospitals. I'm going to be speaking to you on mitochondria in health and disease.
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Mitochondria are essential cellular organelles that, with the exception of red blood cells, occur nearly all eukaryotic cells. The number of mitochondria per cell varies from hundreds to thousands, and is higher in the more metabolically active tissues. The best-known role for mitochondria is to generate energy for the cell, in particular the generation of ATP by oxidative phosphorylation. However, mitochondria may also have roles in other cellular processes such as apoptosis, heat production, amino acid metabolism and lipid metabolism.
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In this slide, we see the organisation of the mitochondria, they're organised into four compartments. There's a smooth outer membrane that is freely permeable to ions and small molecules and an inner folded membrane that encloses the matrix space, the inner membrane folds are known as cristae and they're impermeable to small molecules. The compartment between the inner and outer membranes is known as the intermembrane space. The innermost part (the matrix) contains the mitochondrial DNA and proteins involved in biochemical pathways, such as the tricarboxylic acid or Krebs cycle pathway. Within the matrix, the multimeric complexes of the respiratory chain convert energy derived from nutrients into ATP, in a process known as oxidative phosphorylation. More than 1,500 proteins are required for mitochondria to function.