Outline
• Mitochondrial structure
Aerobic Respiration and the
Mitochondrion • The mechanism of ATP production in mitochondria
• Semiautonomy and the origin of mitochondria and
chloroplasts
1. Mitochondrial morphology
I. Mitochondria and the oxidative
phosphorylation
Figure 14-6 Molecular Biology of the Cell (© Garland Science 2008)
Figure 5-1b Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
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Mitochondria: an extensive network or reticulum 1.1 The fusion and fission of mitochondria
In a mammalian fibroblast, the mitochondria (green) do not appear as discrete,
individual structures but rather as an extensive network or reticulum that
extends through much of the cell.
Figure 5 Cell and Molecular Biology (© John Wiley & Sons, Inc 2010) Figure 14-56a Molecular Biology of the Cell (© Garland Science 2008)
Possible relationship between mitochondrial fusion, fission, biogenesis
1.1 The fusion and fission of mitochondria and degradation.
Seo A Y et al. J Cell Sci 2010;123:2533-2542
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The dynamics of fusion and fission controls the amount of mitochondria
within cells
1.2 The dynamic organelle
Mitochondria are dynamic
organelles that change
their shape, move from
place to place within the
cytoplasm.
Table 14-2 Molecular Biology of the Cell (© Garland Science 2008) Figure 14-4 Molecular Biology of the Cell (© Garland Science 2008)
1.3 Relationship between mitochondria and microtubules 2. Mitochondrial ultrastructure
• The outer
membrane
• The inner
membrane
• The intermembrae
space
• The mitochodrial
matrix
Mitochondria tend to be aligned along microtubules,
which determines their orientation and distribution in
different cell types
Figure 5-3c Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
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Cristae are present as flattened sheet Interaction of mitochondria with ER
Three-dimensional reconstruction of a mitochondrion based on a series of
micrographs taken with a high-voltage electron microscope of a single thick
section of brown fat tissue that had been tilted at various angles. The results
suggest that the cristae are present as flattened sheets (lamellae) that
communicate with the intermembrane space by way of narrow tubular openings,
rather than “widen open” channels as is typically depicted.
Figure 5-3a,b Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
2.1 the outer membrane: permeable 2.2 the inner membrane: impermeable
• A large channel-forming protein: porin (can undergo reversible • The electron-transport chain: the oxidation reactions
closure in response to conditions within the cell.) • The ATP synthase: make ATP in the matrix
• Permeable to all molecules of 5000 daltons or less (ATP, NAD,
• Transport proteins: allow the passage of metabolites into and out
and coenzyme A)
of the matrix
• Other proteins include enzymes involved in mitochondrial lipid
synthesis and enzymes that convert lipid substrates into forms • Inner membrane is devoid of cholesterol and rich in unusual
that are subsequently metabolized in the matrix, import receptors phospholipid, cardiolipin. Both of them are characteristics of bacterial
for mitochondrial proteins, and enzymatic machinery for division plasma membranes.
and fusion of the organel le.
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2.3 the mitochondrial matrix 2.4 the intermembrane space
• The large internal space contains a highly • The space contains several enzymes that use
concentrated mixture of enzymes. the ATP passing out of the matrix to
phosphorylate other nucleotides.
• The matrix has several identical copies of the
mitochondrial DNA genome, special mitochondrial
ribosomes, tRNAs, and various enzymes required
for expression of mitochondrial genes
Cold？ 3. The oxidative phosphorylation
An overview of carbohydrate metabolism in eukaryotic cells
Figure 5-5 Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
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3. The oxidative phosphorylation 3. The oxidative phosphorylation
When ATP formation is driven by energy that is released from electrons removed
during substrate oxidation, the process is called oxidative phosphorylation.
• The electron-transport chain
• The proton motive force
• The ATP synthase
• The impermeability of the inner membrane
Figure 14-14 Molecular Biology of the Cell (© Garland Science 2008)
3.1 The electron-transport chain 3.1 The electron-transport chain
Electron carriers Electron-transport complexes
• Flavoproteins • Complex I: NADH dehydrogenase
• Cytochromes • Complex II : succinate dehydrogenase
• Copper atoms • Complex III: cytochrome bc1
• Complex IV: cytochrome c oxidase
• Ubiquinone (coenzyme Q)
• Iron-sulfur proteins
• Both cytochrome c and ubiquinone are
The diagram illustrates the
necessary parts of this chain, but they are
approximate redox potential of
not part of any the four complexes and
the carriers and the decline in
exist independently within the inner
free energy as electron pairs
membrane.
move along the respiratory
chain to oxygen.
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The electron-transport chain of the inner mitochondrial membrane 3.2 The proton-motive force
The proton-motive force is tend to drives H+ into
the matrix space.
Figure 5-17a Cell and Molecular Biology (© John Wiley & Sons, Inc 2010) Figure 14-13 Molecular Biology of the Cell (© Garland Science 2008)
The proton leak The proton leak
• 2,4-dinitrophenol (DNP): a diet pill ?! • UCPs: abundant in brown adipose cells, skeletal muscle cells (basal metabolic rate,
weight-loss drug), and cold-adapted animals, hibernating animals, newborn animals.
• Uncoupling proteins (UCPs or thermogenin): • Mice that lack UCP1 cannot maintain their body temperature in cold conditions,
whereas normal animals produce larger amounts of UCP1 when they are cold
adapted.
Alaskan Brown Bear
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3.3 ATP synthase 3.3 ATP synthase
• Head: coupling factor 1 (F1),
α3 β3γεδ
• Base: coupling factor 0 (F0,),
ab2c10～12
Walker and colleagues : 1994, 2.8Å, beef heart F1—ATP
Schematic diagram of the ATP synthase from E . coli
synthase crystal, the Nobel Prize in Chemistry, 1997
Figure 5-23a Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
The ATP formation according to the Binding Change Mechanism Experimental demonstration of rotation of Fo and γ
Rotational catalysis
Boyer: the Nobel Prize in Chemistry 1997
From Yoh Wada et al Biochem. [Link] 1459:503,2000
Figure 5-27 Cell and Molecular Biology (© John Wiley & Sons, Inc 2010)
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Functions of ATP synthase Other roles for the proton-motive force in addition to ATP synthesis
Figure 14-19 Molecular Biology of the Cell (© Garland Science 2008) Figure 14-16 Molecular Biology of the Cell (© Garland Science 2008)
4. Mitochondria and diseases Mitochondrial Inheritance
mitochondrial disease All mitochondria inherited from
your mother
• During fertilization mtDNA is derived only
from the oocyte
• Maternal inheritance: mtDNA mutations
transmitted only from mother
• Mutations transmitted to all offspring
Symptoms of Mitochondrial Disease
(male or female)
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Functions of mitochondria
• Generate most of the ATP that is used to run most of the
cell’s energy-requiring activities. II. Semiautonomy and the origin of
• Mitochondria are the sites of synthesis of numerous
mitochondria
substances, including certain amino acids and the heme
groups.
• Mitochondria also play a vital role in the uptake and
release of calcium ions.
• The process of cell death is also regulated to a large
extent by events that occur within mitochondria.
1. The semiautonomy of mitochondria and chloroplasts 2. The origin of mitochondria and chloroplasts
• Endosymbiont hypothesis
– Purple bacteria: mitochondria
• DNA
– Cyanobacteria: chloroplasts
• Ribosomes
• DNA replication
• Protein synthesis
Figure 14-66 Molecular Biology of the Cell (© Garland Science 2008) Figure 12-4b Molecular Biology of the Cell (© Garland Science 2008)
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