The mitochondria is a double membrane-bound organelle found in eukaryotic cells that generates energy in the form of ATP through aerobic respiration. It has an outer and inner membrane that create compartments like the intermembrane space. The inner membrane folds inward forming cristae to increase surface area for oxidative phosphorylation. In addition to producing energy, mitochondria perform other functions like storing calcium, generating heat, and regulating cell growth and death. Mitochondrial diseases occur when the organelles fail to produce sufficient energy for normal cell functioning.
2. MITOCHONDRIA
The mitochondria is a double membrane- bound organelle found
eukaryotic cells, mito= thread chondrion = granule likE
Called Power house of cell and found in cytoplasm of the cell.
Firstly observed by Richard Altman (1894) and term
mitochondria was coined by Carl Benda (1898)
The primary function of which is to generate large quantities of
energy in the form of adenosine triphosphate (ATP) to fuel the
cells activities. The function known as aerobic respiration is the
reason mitochondria are frequently referred to as the powerhouse
of the cell. Aerobic respiration involves: Glycolysis, Krebs cycle
In addition to producing energy, mitochondria
store calcium for cell signaling activities, generate heat, and
mediate cell growth and death.
4. SIZE AND MORPHOLOGY
DIAMETER: Mitochondria range from 0.5 to
1.0 亮m in diameter.
SHAPE: normally Sausage shaped, In
fibroblasts-elongated and thread like
NUMBER: Depends on type size and
functional state of cell. Eg: An average liver
cells contain around 1500 mitochondria.
LOCATION: Cells with high energy
requirements. Eg: Sperm tail, Muscle, Flagella
5. STRUCTURE MITOCHONDRIA DIVIDED INTO FIVE PARTS
1. Outer Membrane 2. Intermembrane Space
3. Inner Membrane 4. Cristae
5. Matrix
They are double membrane bound organelle with their own
DNA and ribosomes.
Outer Membrane- covers the organelle, freely permeable.
Inner Membrane- contains extensive folding called cristae.
Intermembrane space between outer & inner membrane.
Contains cytoplasm.
Matrix- within the Inner Membrane. Contains various
proteins and enzymes.
7. INNER MEMBRANE
Simple phospholipid bilayer. Its
encloses the mitochondrian.
Contain large number of integral
protein structures called porins,
which allows molecules to freely
diffuse from one side of the
membrane to the other.
Porins pass molecules less then
5000 D.
Ions, nutrient molecules, ATP,
ADP etc can pass through the outer
membrane with ease.
The outer mitochondrial
membrane is composed of about
50%
INTER MEMBRANE SPACE
It is also known as
Perimitochondrial space.
It is a space between inner
membrane and outer membrane,
is approximately 70 A.
It has high proton concentration.
Because the outer membrane is
freely permeable to small
molecules, the concentration of
small molecules such as ions and
sugars in the intermembrane
space is same as that of the
cytosol.
Proteins present, participate in
ATP synthesis
8. INNER MEMBRANE
Is freely permeable only to oxygen,
CO , HO.
The inner mitochondrial membrane
contains proteins that perform
redox reactions in oxidative
phosphorylation, ATP synthase,
transport proteins, protein import
machinery, mitochondria fusion
and fission protein.
Several antiport systems exist ,
allowing exchange of anions
between the
cytosol and the
mitochondrial
CRISTAE
Are folds of inner
mitochondrial membrane,
which expand its surface
area , enhancing its
ability to produce ATP.
Stalked particles or inner
membrane spheres :
cristae is covered with
this inner membrane
spheres called stalked
particles or knobs or
heads.
9. MATRIX
It is the space enclosed by the inner membrane.
Gel like consistency, Dense, homogenous.
Contains 2/3 rd of total protein of mitochondria.
Matrix have enzymes, DNA genome, ribosomes,
t-RNA, granules, fibrils and tubules.
The matrix is important in the production of ATP
with the aid of the ATP synthase contained in the
inner membrane.
Major enzymes include enzymes involved in
Synthesis of nucleic acid and proteins, fatty acid
oxidation, TCA cycle
10. FUNCTIONS
Aerobic respiration Mitochondria uses complex molecules and
oxygen to produce a higher energy molecule known as ATP.
Mitochondria are more abundant in the cell that contain a lot of
energy. Eg: muscle.
Extra mitochondrial inheritance: mt-DNA contains plasma genes.
Synthesis of mt-DNA , RNA, protein.
Bring about gene expression through gene duplication transcription
and translation.
Synthesis 13 different polypeptides in human.
Production of heat (non shivering thermogenesis).
Role in apoptosis (programmed cell death).
Synthesis of estrogen and testosterone.
Role in neurotransmitter metabolism.
Role on cholesterol metabolism.
Role in heme synthesis.
11. SITE OF SEVERAL METABOLIC REACTIONS
OUTER
MEMBRANE
Oxidation of
epinephrine
Degradation
of tryptophan
Elongation of
fatty acid
Krebs cycle
Beta oxidation
Detoxification
of ammonia in
urea cycle
Storage of
calcium ions
MATRIX
Oxidative
phosphorylati
on
INNER
MEMBRANE
12. MITOCHONDRIAL DISEASE
This diseases occurs due to
failure of Mitochondria.
Its dysfunction causes
failure in production of
energy necessary to sustain
life.
Primarily occurs in children.
The entire body system can
fail, if the process happens
throughout the body.
Symptoms include: loss of
motor control, muscle
weakness, poor growth,
respiratory illness,
susceptibility to other
diseases.