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Mitochondrial disorders and neurodegeneration
Published on July 31, 2019 42 min
A selection of talks on Cell Biology
Gene structure, expression and regulation: DNA structure and replication
- Dr. Carole Sargent
- University of Cambridge, UK
Preclinical translation of mesenchymal stem cell therapies
- Dr. Peter Childs
- University of Strathclyde, UK
My name is Jan-Willem Taanman, I'm at the Department of Clinical Neurosciences of the Institute of Neurology at University College London. This lecture is on Mitochondrial Disorders and Neurodegeneration.
So in this lecture, I will first discuss the mitochondrial structure, function, and genetics. Then I will give an overview of diseases caused by mutations in mitochondrial DNA, and finally, I will give an overview of diseases caused by mutations in nuclear genes coding for mitochondrial proteins.
Mitochondria are essential eukaryotic organelles. They are the descendants of alphaproteobacteria that formed an endosymbiotic relationship with ancestral eukaryotic organisms. Mitochondria come in different sizes and shapes, but often form a reticular network as shown here in this cultured multi-nuclear myotube. Well, mitochondria are not static but are highly dynamic organelles that undergo continual fission and fusion.
Structurally, mitochondria are characterized by a double membrane; an outer membrane and an inner membrane that demarcate the intermembrane space, and the inner membrane protrudes into the matrix to form the cristae membranes.
Well, per definition, all mitochondria are able to carry out two functions, and that is generation of ATP coupled to electron transport in a process called oxidative phosphorylation. Secondly, the expression of an integral genome. In other words, mitochondria have their own mitochondrial DNA.