MITOCHONDRIA

1. Concept
Energy of the cell, multistep process, the mitochondria are the metabolic masters of
the cell.
Organelle, both animal and vegetal
Main function: produce energy in the form of ATP, has a high energy point that when
it’s broken it realize energy. Mitochondria can be observed in vivo stains and with the
janous green, an stain that have a green colour when its oxidized, and no color when
its reduced. Those spots have a red______- of observation. The size of mitochondria is
from 0.5 microm to 1 microm. And the length from 7 microms up.
The location is really viarable and depends on the type of cells. Asociated to
microtubules, on the cardiac muscle are in fixed positions btw the fibres.
Using microcinematography, we can see that they are very plastic, they change
dpending on the _____ These changes of size means that the size of the mit are
directed related to the power generation. It can go from 1 to have thousands. The
important thing is not the number, bt the relative volum btw the mithocondria and the
total volum of the cell. These volum can be regulated relating to the energy
dependence of the cell

2. General characteristics

3. Ultraestructure
Mit are organized quite simply:
The energy is perfom in the outer membrane. 6nm, and have a space of a thickness of
10nm. The main difference between the outer and inner membrane is that the outer is
continous and the inner has some cresta to increase the surface. The cuantitie is
around 200-400 units in the membrane (lollipops). In the matrix we’ve ribosomes,
granuls and mitochondria dna. All these elements are observed using particular stanes
on microscope.

4. Chemical components
Outer membrane: the chemical comp of the mit was the first reported of the organels.
It have and oxidative environment. The 1 st organelle isolated in the history. More
similar to the cell membrane, 40% lipids 60% proteins, lipids common to the cell
membrane; fosfatidil colin, not too much collesterol, and transportive proteins: porin
(basic transport proteins, a hole, that allows free transport of anything smaller of 500
daltoms), enzymes (for active transport)
The inner membrane: many less lipids and many more proteins. Lipids: cardiolipin the
most abundant lipid that has 4 fattyacid chains. These make the inner membrane
particularly not permeable, not allows passing more molecules on the outer
membrane. No cholesterol. The protein composition is very specifical, we cannot find
that proteins anywhere else. Components of the respiratory chain: nadh 8000 daltons,
cyocrhome b-c1 units 5000 daltos, 8 subunits, cytochrome oxidase complex 3000
 daltons,15 proteins. thes components are essential for the live of the cell. atp
synthasem: 9 subunits, they are the lollypop. Specific transporters: to allow pass to the
matrix. The crebs cicle express on the matrix. These transportes are specific
transportes, for example the adp/atp transporter puts in an atp molecule at the same
time that puts out an adp molecule.
The energy produce by the mit is released in a multistep process. A high energy
electron. The electron lose energy along the chain. These proton gradient is an
electrochemical gradient. This electric prtons go back to the matrix through the ATP
synthase and produce ATP. These is the way these energy is converted in ATP. When
adp molecule is put inc ontact with a subunit of the atp synthase, atp is produced.
When you pass protons, the atp synthase rotates a little bit.
Intermembran space: adenilkinase (AMP + ATP --> 2ADP) . Not so many another things,
the important role is to hole the proton gradient.
Matrix: mithocondrial DNA: doble chain. Circular and close. Not simetrical chains.
Fixed numbers of basepairs. Genetic code different to universal code (than nuclear
DNA) Codifies 13 pp, 22 tRNA and 2 rRNA. A particular situation for these dna is less
stable than the cell dna. Not protected by histones. Not fixing molecules.

Ribosomes
tRNAs
Enzymes for oxidations
Enzymes related to DNA activity.
They have all the tools to produce protein material.

5. Functions
1) Respiratory oxidations:
-acetil CoA formatted on the matrix.
-acetil-CoA oxidation (krebs cycle) on the matrix.
-electron transport and phosphorilation
-Quimiosmosis

2) Generation of precursors for gluconeogenesis, fatty acid synthesis…

3) Protein synthesis: it has all the tools. Bt it only produce 15 proteis (less than 1% of
the proteins that the mithocondria needs)
The other proteins that it needs, comes from the cytoplasm. TIM/TOM complex
4) Apoptosis: one of the 1st steps in apoptosis is the released of citochrome in the
inner space of the mit.

6. Biogénesis
They are always generated by other mitochondria, by fusion. Mitochondria dna is only
inherited from the molecule. Mitocondria dna comes from the mother.
Pathology: aging: is accumulative procces of errors in the dna, that come from the
oxidative species generated on the mit.
Mit pathologies affect to the organs that need more energy: encephalopathies and
myopathies.