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- Cellular Mechanisms of Mitochondrial Biogenesis in Health
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1. Nuclear control of electron transport chain gene expression
- Dr. Glenn C. Rowe
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3. Protein import into mitochondria
- Dr. Trevor Lithgow
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4. Mitochondrial phospholipid synthesis and incorporation
- Prof. Grant Hatch
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5. Mitochondrial DNA replication and transcription
- Prof. Julio Montoya
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6. Mitochondrial production of reactive oxygen species 1
- Prof. Martin Brand
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7. Mitochondrial production of reactive oxygen species 2
- Prof. Martin Brand
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8. Mitophagy - the degradation of dysfunctional organelles
- Dr. Anna Vainshtein
- Mitochondrial Biogenesis in Physiological Conditions
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9. Thyroid hormone and mitochondrial biogenesis
- Dr. Joachim Weitzel
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10. Exercise-induced mitochondrial biogenesis in muscle 1
- Prof. David Hood
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11. Exercise-induced mitochondrial biogenesis in muscle 2
- Prof. David Hood
- Mitochondrial Biogenesis in Aging and Disease
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12. Mitochondrial diseases: an update
- Dr. Ayesha Saleem
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13. Exercise and nutrition in mitochondrial disorders
- Prof. Mark Tarnopolsky
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14. Mitochondrial disorders and neurodegeneration
- Dr. Jan-Willem Taanman
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15. Reprogramming oxidative phosphorylation in cancer
- Prof. José Cuezva
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16. Possible roles of mitochondrial biogenesis in aging
- Dr. Aubrey de Grey
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17. Mitochondria in reproduction and fertility: mitochondria and gametes 1
- Prof. Pascale May Panloup
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18. Mitochondria in reproduction and fertility: mitochondria and embryo 2
- Prof. Pascale May Panloup
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19. Mitochondrial morphology and ultrastructure in skeletal muscle
- Prof. Martin Picard
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20. Improving mitochondrial phenotypes with pharmaceuticals
- Prof. Christopher Perry
- Mitochondria, Cell Signaling and Apoptosis
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21. Mitochondrial permeability transition
- Prof. John Lemasters
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22. Pathways of apoptosis in muscle 1
- Prof. Stephen E. Alway
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23. Pathways of apoptosis in muscle 2
- Prof. Stephen E. Alway
- Archived Lectures *These may not cover the latest advances in the field
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26. Reactive oxygen species and myocardial apoptosis
- Dr. Zhi-Qing Zhao
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27. Mitochondrial biogenesis as a result of exercise
- Dr. Darrell Neufer
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28. Mechanisms of mitochondrial fusion and fission
- Dr. Luca Scorrano
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29. Mitochondrial heterogeneity in cells
- Dr. Tony Collins
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30. Mitochondrial diseases: an update
- Prof. Salvatore DiMauro
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31. Calcium signaling and mitochondrial function
- Prof. David Nicholls
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32. Mitochondrial biogenesis during gametogenesis and embryonic development
- Prof. Pascal Reynier
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33. Mitochondrial complex assembly
- Dr. Leo Nijtmans
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34. Evolutionary and developmental variation in muscle mitochondrial content
- Prof. Christopher Moyes
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35. Muscle mitochondrial function and biogenesis with aging
- Dr. Russell Hepple
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36. The mitochondrial pathway in apoptosis
- Prof. Douglas Green
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38. Mitochondrial disorders and neurodegeneration
- Prof. Anthony Schapira
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39. Respiration, reactive oxygen species and uncoupling proteins
- Prof. Martin Brand
Printable Handouts
Navigable Slide Index
- Introduction
- Mitochondrial disorders and neurodegeneration (1)
- Mitochondria- essential eukaryotic organelles (1)
- Mitochondria- essential eukaryotic organelles (2)
- Mitochondria- essential eukaryotic organelles (3)
- Mitochondrial DNA (mtDNA)
- Mitochondrial proteins
- Bioenergetic pathways
- The mitochondrial OXPHOS enzyme system
- The mitochondrial OXPHOS enzyme complexes
- The L and H strands
- The mtDNA D-loop
- Replication of mtDNA
- BrdU detects DNA replicating DNA
- mtDNA replication is independent of the cell cycle
- Initiation of mtDNA replication
- mtDNA shows maternal inheritance
- Maternal inheritance of mtDNA molecular basis
- Mitochondrial disorders and neurodegeneration (2)
- mtDNA mutations (1)
- mtDNA mutations (2)
- The m.3243A>G mtDNA point mutation (1)
- The m.3243A>G mtDNA point mutation (2)
- The m.3243A>G mtDNA point mutation (3)
- Mutations result in a decrease of ATP production
- mtDNA mutations increrase ROS production
- mtDNA point mutations show maternal inheritance
- ART prevent transmission of mtDNA diseases (1)
- ART prevent transmission of mtDNA diseases (2)
- Mitochondrial disorders and neurodegeneration (3)
- Nuclear gene defects in mitochondrial diseases (1)
- Nuclear gene mutations & defects of mtDNA (1)
- Nuclear gene mutations & defects of mtDNA (2)
- Alpers’ disease caused by POLG mutations
- Nuclear gene mutations & defects of mtDNA (3)
- Defects of mt deoxynucleotide synthesis
- BrdU incorporation into replicating DNA
- Southern blot analysis of quiescent fibroblasts
- Nuclear gene defects in mitochondrial diseases (2)
- Friedreich’s ataxia – clinical hallmarks
- Friedreich’s ataxia – pathogenesis
- Nuclear gene defects in mitochondrial diseases (3)
- Hereditary spastic paraplegia – clinical hallmarks
- Hereditary spastic paraplegia – pathogenesis
- Nuclear gene defects in mitochondrial diseases (4)
- Nuclear gene defects in mitochondrial diseases (5)
- Mitochondrial fission and fusion
- Nuclear gene defects in mitochondrial diseases (6)
- Parkinson’s disease – clinical hallmarks
- Parkinson’s disease – pathological hallmarks
- Parkinson’s disease – pathogenesis
- Induction of mitophagy by PINK1 and Parkin
- Mitochondrial disorders and neurodegeneration (4)
Topics Covered
- Mitochondrial structure and function
- Oxidative phosphorylation
- Mitochondrial DNA (mtDNA) structure, replication and maternal inheritance
- Diseases caused by mutations in mtDNA and therapy
- Diseases caused by mutations in nuclear genes coding for mitochondrial proteins
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Taanman, J. (2019, July 31). Mitochondrial disorders and neurodegeneration [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved May 11, 2025, from https://doi.org/10.69645/THJF1733.Export Citation (RIS)
Publication History
- Published on July 31, 2019
Financial Disclosures
- Dr. Jan-Willem Taanman has received consulting fees/honoraria from Novintum Biosciences Ltd., royalties from Abcam, and research support from The Michael J Fox Foundation.
A selection of talks on Biochemistry
Transcript
Please wait while the transcript is being prepared...
0:00
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.
0:16
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.
0:40
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.
1:16
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.
1:33
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.