Mitochondria Modified

Presented by
Janvi parekh
HISTORY
 It raised about 2 billion years ago when a bacterium
fused with an archaeal cell.
 The closest extinct relative of the bacterium that gave
rise to mitochondria is rickettisia.
 Rickettsia is an aerobic α-proteobacteria with a
genome of 1.1 megabase.
 but it is an intracellular pathogen and causes a
diseases called rocky mountain spotted fever.
 It is believed that mitochondrial was engulfed by the
early eukaryotic cell but could not digest it, so it
remained as an endosymbiont and established itself
into the cell.
 It is proved by endosymbiosis theory that
mitochondria, chloroplast would have entered
the early eukaryotic cell .
 Mitochondria has it own mitochondrial genomes
which is known 16sRNA.
 But during the course of evolution some genes were
mutated and went to nucleus and so it cannot live
without host.
DISCOVERY OF MITOCHONDRIA
VON KOILLIKER CARL BENDA
DISCOVERY
 It was first described by a the Swiss anatomist and

physiologist “VON KOILLIKER” in 1856.
 He saw it during when he was studying muscle tissue
and termed as sacrosomes.
 It was later termed as mitochondria by Benda in 1898
when he observed the organelles during
spermatogenesis.
 In Greek ‘mito’ means ‘thread’ and ‘chondros’ means
‘granules’.
MORPHOLOGY AND STRUCTURE
OF MITOCHONDRIA
MORPHOLOGY
 SIZE:- mitochondria ranges form 0.5 to 1.0µm in
diameter.
 SHAPE:-normally sausage shaped.
In fibroblast elongated and thread like
 NUMBER:- depends on type size an functional state
of cell.
Eg:- an average liver cells contains around 1500
mitochondria.
 LOCATION:- cell with high energy requirements
Eg:-sperm tail, muscle, flagella.
STRUCTURE
 It is double membrane bound organelles found in

eukaryotic cell.
 It consist of the structure like:-
Inner MEMBRANE
OUTER MEMBRANE
INTMEMBRANE SPACE
CRISTAE
MATRIX
OUTER MEMBRANE
 Simple phospholipid bilayer.
 It encloses the mitochondrion.
 Containing protein structures called porins.
 Ions, nutrient molecules, ATP, ADP, etc. can pass
through the outer membrane with ease.
INNER MEMBRANE
 Is freely permeable ONLY to oxygen, carbon
dioxide and water.
 Contains complexes of the electron transport chain
(ETC), the ATP synthase complex and transport
proteins.
 Presence of sophisticated ion transporters exist.
 Several antiport system exist, allowing exchange of
anions between the cytosol and the mitochondrial
matrix.
 It is devoid of cholesterol and rich in phospholipid &
cardiolipin.
INTERMEMBRANE SPACE
 The space between inner membrane and outer

membrane.
 It has high proton concentration .
 The space between inner and outer membrane is

approx. 70 angstrom.
 Concentration is same as that of the cytosol

CRISTAE
 They are folds of inner mitochondrial membrane.
 Stalked particles or inner membrane spheres: cristae is

covered with this inner membrane spheres called
stalked particles or knobes or heads.
 They contain protiens called F1 protein and F0 protien.

For ATP synthesis and H2O oxidation.
MATRIX
 Gel like consistency, dense, homologous.

 2/3rd of total protein of mitochondria
 It has enzymes, DNA, mRNA, granules, fibrils, tubules.
 Major enzymes include enzymes include in:-
Synthesis of nucleic acid and proteins
Fatty acid oxidation
Tricarboxilic acid (TCA) CYCLE(except succinate
dehyrogenase)
FUNCTIONS OF MITOCHONDRIA
OXIDATIVE PHOSPHORYLATION
 It is the process in which ATP is formed as a result of the
transfer of electrons from NADH or FADH2 to O2 by a
series of electron carriers.
 It take place in mitochondria which is major source of ATP
in aerobic organisms .
 The NADH and FADH2 formed in glycolysis, fatty acid
oxidation, and the citric acid cycle are energy-rich
molecules because each contains a pair of electrons having
a high transfer potential.
 When these electrons are used to reduce molecular oxygen
to water, a large amount of free energy is liberated, which
can be used to generate ATP.
 Oxidative phosphorylation is conceptually simple and
mechanistically complex. Indeed, the unraveling of the
mechanism of oxidative phosphorylation has been one of
the most challenging problems of biochemistry.
 The flow of electrons from NADH or FADH2 to O2 through
protein complexes located in the mitochondrial inner
membrane leads to the pumping of protons out of the
mitochondrial matrix.
 The resulting uneven distribution of protons generates a
pH gradient and a transmembrane electrical potential that
creates a proton-motive force.
 ATP is synthesized when protons flow back to the
mitochondrial matrix through an enzyme complex.
 Thus, the oxidation of fuels and the phosphorylation
of ADP are coupled by a proton gradient across the inner
mitochondrial membrane.
Kreb’s cycle and oxidative phosphorylation in
mitochondria
 The mechanism by which mitochondria make ATP is
known as chemosmotic model. It is proposed by
Dr. Peter Mitchell
 He introduced a radical proposal in 1961 to explain the
mechanism by which mitochondria make ATP. It is known
as the chemiosmotic hypothesis and has been shown
over the years to be correct.
 Mitchell proposed that synthesis of ATP in mitochondria
depends on an electrochemical gradient, across the
mitochondrial inner membrane, that arises ultimately from
the energy of reduced electron carriers, NADH and
FADH2.
What happens if mitochondria is
absent in cells ?
 Without mitochondria higher animals would likely not
exist because their cells would only be able to obtain energy
from anaerobic respiration (in the absence of oxygen), a
process much less efficient than aerobic respiration.
 The number of mitochondria per cell varies widely; for
example, in humans, erythrocytes (red blood cells) do not
contain any mitochondria, whereas liver cells and
muscle cells may contain hundreds or even thousands. The
only eukaryotic organism known to lack mitochondria is
the oxymonad Monocercomonoides species.
 In fact, mitochondria enable cells to produce 15 times
more ATP than they could otherwise, and complex
animals, like humans, need large amounts of energy in
order to survive.
We get mitochondria from our
mother only…..
MITOCHONDRIAL DISESASES
 The term “Mitochondrial disease” refers to a broad range
of disorders, each of which involves a mitochondrial
dysfunction, with many more believed to have yet to be
discovered.
 Because mitochondria perform so many different functions
in different tissues, there are literally hundreds of different
mitochondrial diseases that result from either inherited or
spontaneous mutations in mtDNA or nDNA, which lead to
altered functions of the proteins or RNA molecules that
normally reside in mitochondria.
 Another subcategory is Mitochondrial myopathies — a
group of neuromuscular diseases caused by damage to
the mitochondria with some examples including :-
1. Kearns-Sayre syndrome (KSS)
2. Leigh’s syndrome
3. Mitochondrial Depletion syndrome (MDS)
4. Mitochondrial Encephalomyopathy
5. Lactic Acidosis and Stroke-like episodes (MELAS)
6. Myoclonic epilepsy with Ragged Red Fibers (MERRG)
7. Mitochondrial neurogastrointestinal encephalopathy
syndrome (MNGIE)
8. Neuropathy, Ataxia
9. Retinitis Pigmentosa (NARP)
10. Pearson syndrome, and Chronic Progressive External
Opthalmoplegia (CPEO).
 Nerve cells in the brain and muscles require a great
deal of energy and appear to be particularly vulnerable
when mitochondrial dysfunction occurs.
 One in 5,000 individuals has a genetic mitochondrial
disease.
 Each year, about 1,000 to 4,000 children in the United
States are born with a mitochondrial disease.
 With the number and type of symptoms and organ
systems involved, mitochondrial diseases are often
mistaken for other, more common diseases.
Summary

Mitochondria Modified

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 It was first described by a the Swiss anatomist and

 It is double membrane bound organelles found in

 The space between inner membrane and outer

 It has high proton concentration .

 The space between inner and outer membrane is

 Concentration is same as that of the cytosol

 They are folds of inner mitochondrial membrane.

 Stalked particles or inner membrane spheres: cristae is

 They contain protiens called F1 protein and F0 protien.

 Gel like consistency, dense, homologous.

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