Scribd
Upload
35 views29 pages

k2. MItochondria - Muzar Z-Des 2016

Mitochondria are membrane-enclosed organelles found in the cells of eukaryotic organisms that generate most of the cell's supply of adenosine triphosphate (ATP), used as a source of chemical energy. Mitochondria contain their own circular DNA and ribosomes, and can replicate independently of the cell's nucleus. They have an outer and inner membrane which creates compartments and folds in the inner membrane called cristae that increase surface area. Enzymes in the cristae perform the functions of respiration and ATP synthesis through oxidative phosphorylation. ATP is produced as protons flow back through the ATP synthase complex using the energy from the electrochemical gradient created by the electron transport chain. Mitochondrial DNA

Uploaded by

Khemal Mubaraq
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
35 views29 pages

k2. MItochondria - Muzar Z-Des 2016

Mitochondria are membrane-enclosed organelles found in the cells of eukaryotic organisms that generate most of the cell's supply of adenosine triphosphate (ATP), used as a source of chemical energy. Mitochondria contain their own circular DNA and ribosomes, and can replicate independently of the cell's nucleus. They have an outer and inner membrane which creates compartments and folds in the inner membrane called cristae that increase surface area. Enzymes in the cristae perform the functions of respiration and ATP synthesis through oxidative phosphorylation. ATP is produced as protons flow back through the ATP synthase complex using the energy from the electrochemical gradient created by the electron transport chain. Mitochondrial DNA

Uploaded by

Khemal Mubaraq
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
You are on page 1/ 29

Mitochondria

dr. Zukhrofi Muzar, M.Si.Med


dr. Des Suryani, M. Biomed

Department of Histology
Muhammadiyah University of North Sumatera
Faculty of Medicine
2016
Definition
• Mitochondria (Gr. mitos, thread, +
chondros, granule) are membrane-
enclosed organelles with arrays of
enzymes specialized for aerobic
respiration and production of ade-
nosine triphosphate (ATP), with high-
energy phosphate bonds, which
supplies energy for most cellular
activities (powerhouses of cells)
What makes mitochondria
unique?
• Unlike most organelles mitochondria
are partly autonomous of nuclear genes
and activities. The mitochondrial matrix
contains a small circular chromosome
of DNA, ribosomes, mRNA, and tRNA,
all with similarities to the corresponding
bacterial components.
Mitochondria is the
powerhouses of the cells
• Glycolysis converts glucose
anaerobically to pyruvate in the
cytoplasm, releasing some energy.
• Pyruvate is imported into mitochondria
and oxidized to CO2 and H2O producing
the rest of the energy.
• Mitochondrial enzymes yield 15 times
more ATP than is produced by
glycolysis alone.
Structure of mitochondria
• Mitochondria are usually elongated
structures with diameters of 0.5-1 μm
and lengths up to 10 times greater.
• They are highly plastic, rapidly changing
shape, fusing with one another and
dividing, and are moved through the
cytoplasm along microtubules
• Mitochondria
are often large
enough to be
visible with the
light
microscope as
numerous
discrete
organelles.
Structural components
• Under the TEM each mitochondrion is seen to
have two separated and very different
membranes that together create two
compartments: the innermost matrix and a
narrow intermembrane space.
• The inner mitochondrial membrane surrounds
a space called the matrix. The outer
mitochondrial membrane is in close contact
with the cytoplasm. The space between the
two membranes is called the intermembrane
space.
Outer mitochondrial
membrane
• The outer membrane is sieve-like,
containing many transmembrane
proteins called porins that form
channels through which small
molecules such as pyruvate and other
metabolites readily pass from the
cytoplasm to the intermembrane space.
Outer mitochondrial
membrane
• This 6- to 7-nm-thick smooth membrane contains many
voltage-dependent anion channels (also called
mitochondrial porins).
• These large channels (approximately 3 nm in diameter) are
permeable to uncharged molecules as large as 5,000
daltons. Thus, small molecules, ions, and metabolites can
enter the intermembrane space but cannot penetrate the
inner membrane. The environment of the intermembrane
space is therefore similar to that of cytoplasm with respect
to ions and small molecules.
• The outer membrane possesses receptors for proteins and
polypeptides that translocate into the intermembrane
space. It also contains several enzymes, including
phospholipase A2, monoamine oxidase, and acetyl
coenzyme A (CoA) synthase.
Inner mitochondrial
membrane
• The TEM reveals that this membrane is thinner than the outer
mitochondrial membrane.
• It is arranged into numerous cristae (folds) that significantly
increase the inner membrane surface area. These folds project into
the matrix that constitutes the inner compartment of the organelle.
• The inner membrane is rich in the phospholipid cardiolipin, which
makes the membrane impermeable to ions.
• The membrane forming the cristae contains proteins that have three
major functions: (1) performing the oxidation reactions of the
respiratory electron-transport chain, (2) synthesizing ATP, and (3)
regulating transport of metabolites into and out of the matrix. The
enzymes of the respiratory chain are attached to the inner
membrane and project their heads into the matrix.
• With the TEM, these enzymes appear as tennis racquet–shaped
structures called elementary particles. Their heads measure about
10 nm in diameter and contain enzymes that carry out oxidative
phosphorylation, which generates ATP.
Intermembrane space
• This space is located between the inner
and outer membranes and contains
specific enzymes that use the ATP
generated in the inner membrane. These
enzymes include creatine kinase,
adenylate kinase, and cytochrome c.
• In the cytoplasm cytochrome c activates
sets of proteases that degrade all
cellular components in a regulated
process called apoptosis that results in
rapid cell death.
Matrix

• The mitochondrial matrix is surrounded


by the inner mitochondrial membrane
and contains the soluble enzymes of the
citric acid cycle (Krebs cycle) and the
enzymes involved in fatty acid beta-
oxidation.
• The major products of the matrix are
CO2 and reduced NADH, which is the
source of electrons for the electron-
transport chain.
Matrix

• Matrix enzymes include those that


oxidize pyruvate and fatty acids to form
acetyl coenzyme A (CoA) and those of
the citric acid cycle that oxidize acetyl
CoA, releasing CO2 as waste and small
energy-rich molecules that provide
electrons for transport along the
electron- transport chain (or respiratory
chain).
ATP production
• Formation of ATP by oxidative
phosphorylation enzymes occurs by a
chemiosmotic process. Membrane
proteins guide the small electron carrier
molecules through closely packed
enzyme complexes so that the electrons
move sequentially along the chain.
ATP production
• Electron transfer is coupled with oriented
proton uptake and release, with protons
accumulating in the intermembrane space and
producing an electrochemical gradient across
the inner membrane.
• Membrane-associated proteins of the ATP
synthase system form large (10-nm),
multisubunit, globular complexes on stalk-like
structures that project from the matrix side of
the inner membrane.
ATP production
• Through this enzyme complex runs a
hydrophilic pathway that allows protons to ow
down the electrochemical gradient, crossing
the membrane back into the matrix.
• Passage of protons through this channel
causes rotation of specific polypeptides in the
globular ATP synthase complex, converting
the energy of proton flow into the mechanical
energy of protein movement.
ATP production
• Mechanical energy is stored in the new
phosphate bond of ATP by other subunit
polypeptides binding adenosine
diphosphate (ADP) and inorganic
phosphate.
• A steady torrent of protons along the
gradient allows each of these
remarkable synthase complexes to
produce more than 100 molecules of
ATP per second.
• http://www.nature.com/scitable/topicpage/mitochondria-14053590
Inheritance of mitochondrial
DNA
• New mitochondria originate by growth
and division (fission) of preexisting
mitochondria. During cell mitosis each
daughter cell receives approximately
half the mitochondria in the parent cell.
Inheritance of mitochondrial
DNA
• Children inherit their mitochondrial DNA from
their mothers only.
• Although both sperm and egg cells
contain mitochondria, the mitochondria from the
sperm are broken down shortly after fertilisation,
which means that all the mitochondria, and all the
copies of the mitochondrial DNA in the fertilised
egg are from the mother.  This means that since
children inherit their entire mitochondrial
DNA from their mothers it follows that it is only
women that can pass on the mutations within
this DNA that cause disease.
Mitochondrial dysfunction
and diseases
• At least 1 in 5000 individuals being affected
by mitochondrial dysfunction and disease
(Schaefer et al 2008).
• Mitochondrial dysfunctions are associated
with a large proportion of human diseases,
such as neurodegenerative disorders,
cardiovascular disorders, neurometabolic
diseases, cancer, obesity, etc
Additional sources
• http://www.newcastle-mitochondria.com
/patient-and-public-home-page/about-mi
tochondria
/
• http://
www.nature.com/scitable/topicpage/mit
ochondria-14053590
• http://www.umdf.org/site/c.8qKOJ0MvF7
LUG/b.7934629/k.4C9B/Types_of_Mitoch
ondrial_Disease.htm
Additional sources
• https://
ghr.nlm.nih.gov/mitochondrial-dna#con
ditions
• https://www.ncbi.nlm.nih.gov/books/NB
K1224/
Homework
• Buat ringkasan review article Indian J
Med Res 141, January 2015, pp 13-26
• Tugas ditulis tangan, dibuat pada kertas
HVS, 1 halaman penuh
• Nilai tugas = +30 nilai pretest
• Thanks

You might also like