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Mitochondria PDF

Cellular respiration takes place in the mitochondria. Mitochondria are oval-shaped organelles found in the cytoplasm of eukaryotic cells, particularly near structures that require a lot of energy. They contain a smooth outer membrane and inner membrane with folds called cristae. The space within the inner membrane is called the mitochondrial matrix. It is here where the enzymes of the citric acid cycle and electron transport chain are located to produce ATP through oxidative phosphorylation, the process that harnesses energy from food. Mitochondria are involved in many diseases when this energy production is disrupted.

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533 views

Mitochondria PDF

Cellular respiration takes place in the mitochondria. Mitochondria are oval-shaped organelles found in the cytoplasm of eukaryotic cells, particularly near structures that require a lot of energy. They contain a smooth outer membrane and inner membrane with folds called cristae. The space within the inner membrane is called the mitochondrial matrix. It is here where the enzymes of the citric acid cycle and electron transport chain are located to produce ATP through oxidative phosphorylation, the process that harnesses energy from food. Mitochondria are involved in many diseases when this energy production is disrupted.

Uploaded by

CafassoAndrea
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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-Where the cellular respiration takes place.

A.C.
A.G.
A.D.
E.M.
Location :
• Their location is not fix, they are near
the structures that required high
energy deposits

• We find them in Eukaryotic cells, in a


specific way in the cytoplasm.

• They are not present in Red blood


cells
Structure:
-Mitochondria are between 3-10μm in length, and often form branched
networks within cells.
-The outer phospholipid membrane is smooth, making the
mitochondrion ‘bean-shaped’.
-They have a permeable membrane that helps the aerobic respiration
happen.
-Their volumes can change easily, it depends from their hydration.
-They have high plasticity
Ultrastructure:
The external membrane:

• It's rich in phospholipids, enzymes, proteins and cholesterol


• thin, smooth and semipermeable
• it separates the mitochondria from the cytosol
• permeability is due to the presence of channel proteins called porins
The internal membrane:

• it's composed by proeins and lipids


• Same thickness as the external membrane
• it has a complex structures with mitochondrial cristae, they can be
villous, tubular, septal etc.
• mitochondrial cristae are organized perpendicular to the long axis of
the mitochondrion
• He described the crestae, structures which are called
elementary particles or oxidosomes, present on the
surface of the internal membrane

• Oxidosomes are divided in 3 parts :


I. a basal piece that links to the internal membrane
II. a stalk 3 nm long
III. a spherical part called cephalic
• One of the functions of the mitochondria is the relase of ATP into the
matrix, where ATPase is located

• The ATPase complex forms the mitochondrial repiratory chain with


the ion transporters

• Enzymes as adenylate kinase, creatine and diphosphokinase are


located in the intermembrane area
• in the intermembrane area oxidative phosphorization occurs that is
the syntesis of ATP by phosphorization of ADP

• Adenylate kinase catalizes -> ATP + AMP -> 2ADP

So that ATP synthesis occurs thanks to ADP and AMP is reused


• Has a granular structures (calcium deposits -> Mitocondrion is involved in
the capture and release of
calcium ions needed muscle
contraction).

• contains small mitochondrial ribosomes, tRNA, the mitochondrial


genome and enzymes -> pyruvate and fatty acid oxidation to acetyl
coenzyme A, the Krebs cycle and DNA, RNA
and proteins synthesis
• the mithocondrial DNA it's not protected, it has circular shape and is
attached to the inner membrane

• The nucleic acid content makes the mithocondrial partially genetically


autonomy, they can indipendently syntetize enzyme proteins without
the helps of the nucleus
• Mitochondria is the location of cellular respiration.

• It's also involved in cellular and biosyntetic energy metabolism so the


energy necessary for any cellular activity is generated

• The organism’s energy support is represented by food intake;


nutrients are broken down into amino acids, glucose, fatty acids

• Thus in the matrix of the mitochondrion the metabolisation of


pyruvate, fatty acid oxidation and the Krebs cycle take place
• Nearly all our cells rely on mitochondria
for a steady energy supply, It may be
considered that mitochondria are
practically involved in all diseases; cell
viability is ensured by the energy
provided by mitochondria
• Leber disease – an optic neuropathy leading to blindness

• MERRF (Myoclonic Epilepsy with Ragged Red Fibers)

• MELAS (Myopathy, Encephalopathy, Lactic Acidosis, and Stroke)

• Leigh syndrome, an acute encephalopathy with fatal end in 2-5 years

• Luft disease which causes hyperthermia and excessive transpiration

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