-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