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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
- Mitochondria, Cell Signaling and Apoptosis
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20. Mitochondrial permeability transition
- Prof. John Lemasters
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21. Pathways of apoptosis in muscle 1
- Prof. Stephen E. Alway
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22. 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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25. Reactive oxygen species and myocardial apoptosis
- Dr. Zhi-Qing Zhao
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26. Mitochondrial biogenesis as a result of exercise
- Dr. Darrell Neufer
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27. Mechanisms of mitochondrial fusion and fission
- Dr. Luca Scorrano
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28. Mitochondrial heterogeneity in cells
- Dr. Tony Collins
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29. Mitochondrial diseases: an update
- Prof. Salvatore DiMauro
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30. Calcium signaling and mitochondrial function
- Prof. David Nicholls
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31. Mitochondrial biogenesis during gametogenesis and embryonic development
- Prof. Pascal Reynier
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32. Mitochondrial complex assembly
- Dr. Leo Nijtmans
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33. Evolutionary and developmental variation in muscle mitochondrial content
- Prof. Christopher Moyes
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34. Muscle mitochondrial function and biogenesis with aging
- Dr. Russell Hepple
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35. The mitochondrial pathway in apoptosis
- Prof. Douglas Green
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37. Mitochondrial disorders and neurodegeneration
- Prof. Anthony Schapira
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38. Respiration, reactive oxygen species and uncoupling proteins
- Prof. Martin Brand
Printable Handouts
Navigable Slide Index
- Introduction
- Outline
- What are mitochondria?
- Exercise-induced mitochondrial biogenesis
- Mitochondrial biogenesis
- Mitochondrial DNA (mtDNA)
- Mitochondrial nucleoid
- The electron transport chain
- Epidemiology of mitochondrial diseases
- Discovery of mtDNA mutations → mitochondrial diseases (1988)
- Discovery of mtDNA mutations → mitochondrial diseases (1992)
- Patterns of inheritance
- mtDNA heteroplasmy
- Adult onset of mitochondrial disease
- Mitochondrial respiratory-chain disease
- Mitochondrial diseases (1)
- Mitochondrial diseases (2)
- MELAS
- LHON
- KSS
- Childhood onset of mitochondrial disease
- Nuclear gene defects in mitochondrial diseases and their function
- Childhood-onset mitochondrial disease
- Mitochondrial diseases (3)
- POLG
- Leigh syndrome
- Clinical symptoms of mitochondrial diseases
- Mitochondrial disease diagnosis
- Treatment strategies
- Mouse model of aging: polymerase gamma (POLG) mutator mice
- Exercise rescues progeroid aging and induces systemic mitochondrial rejuvenation
- Therapeutic options for mitochondrial diseases
- Current clinical trials in mitochondrial diseases
- Other mitochondrial-related disorders
- Summary
- Acknowledgements
- References (1)
- References (2)
Topics Covered
- Mitochondria and mitochondrial biogenesis
- mtDNA and nucleoids
- The electron transport chain
- Epidemiology of mitochondrial diseases
- Heteroplasmy
- Mitochondrial diseases
- Adult and childhood onset of mitochondrial diseases (POLG, MELAS, LHON, KSS, Leigh syndrome)
- Treatment of mitochondrial diseases
- Mitochondrial-dysfunction related disorders
Links
Series:
- Mitochondrial Biogenesis
- Periodic Reports: Advances in Clinical Interventions and Research Platforms
Categories:
Talk Citation
Saleem, A. (2020, March 31). Mitochondrial diseases: an update [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 23, 2024, from https://hstalks.com/bs/4200/.Export Citation (RIS)
Publication History
Financial Disclosures
- There are no commercial/financial matters to disclose.
A selection of talks on Cell Biology
Transcript
Please wait while the transcript is being prepared...
0:00
Welcome to the Henry Stewart Talks.
My name is Ayesha Saleem,
and I'm an Assistant Professor at
the Faculty of Kinesiology and Recreation Management at the University of Manitoba,
and a Principal Investigator at the Children's Hospital Research Institute of Manitoba.
The title of today's talk is mitochondrial diseases: an update.
0:22
This is the outline for the talk today.
First, I will give some background information about
the mitochondria and its structural components.
Then, I will delve into the epidemiology of mitochondrial diseases.
I will follow that up by looking at adult-onset and then
childhood-onset mitochondrial diseases that are characterized
primarily by defects in mitochondrial and nuclear DNA, respectively.
Finally, I will explore the symptoms and
therapeutic options for mitochondrial diseases and finish
the talk by discussing a subset of
non-communicable diseases that are linked to dysfunctional mitochondria.
0:60
So, about two billion years ago,
a single fusion event between
a prokaryotic and a eukaryotic cell may have
ultimately led to the presence of mitochondria within our cells today.
Mitochondria are tiny organelles found in
almost all the eukaryotic cells and are necessary for cell form and function.
Known as the powerhouse of the cell,
mitochondria are responsible for creating more than 90 percent of
cellular energy, or ATP, through oxidative phosphorylation.
In addition to their main role in energy production and metabolism,
mitochondria are the site for synthesis of iron-sulfur clusters,
steroid biosynthesis, and are involved in initiating apoptosis or programmed cell death,
production and removal of reactive oxygen species,
oxidation of fatty acids,
and regulation of calcium signaling.
Shown is a schematic of a mitochondrion.
Mitochondria are made of a double phospholipid layer,
an outer mitochondrial membrane and
an inner mitochondrial membrane that folds upon itself and creates cristae,
thereby maximizing surface area for chemical reactions.
That space between the two membranes is called the intermembrane space,
and the space enclosed by the inner mitochondrial membrane is known as the matrix.
Mitochondria are unique in that they are the only subcellular organelles to have
their own DNA that can be found inside
the matrix in close proximity to the electron transport chain.
Textbook depictions of mitochondria usually resort to a kidney bean-shaped structure.
In reality, mitochondria often exist in the form of
a reticular interconnected network as shown in the electron micrograph.
The dark structures are the mitochondria found inside a skeletal muscle fiber.
You can clearly appreciate the beautiful organization of cristae within
each mitochondrion that gives the mitochondria their characteristic wrinkled appearance.
The number of mitochondria per cell varies depending on the type of the cell.