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Chapter 6

Mitochondrion

Outer membrane
Inner membrane Inter membrane space

Cristae
Matrix ATP synthase particles DNA

Mitochondria provide the energy which a cell needs to move, divide, produce secretory products, in short, they are the power centers of the cell. Mitochondria are about the size of bacteria but may have different shapes depending on the cell type. There are many mitochondria in animal tissues.

Mitochondria are sometimes described as "cellular power plants" because they generate most of the cell's supply of ATP, used as a source of chemical energy. In addition to supplying cellular energy, mitochondria are involved in a range of other processes, such as signaling, cellular differentiation, cell death, as well as the control of the cell cycle and cell growth.

The structure of Mitochondria


Outer membrane

Inner membrane
Intermembrane space Cristae Matrix ATP synthase particles

DNA

The biological character of mitochondrion 1.Discovery and function research of mitochondrion Altmann found it by the light microscope in the animal cell in 1890. Renda give the name of mitochondrion in 1897. in 1948 George Hogeboom adopt saccharose-medium technology to get activated mitochondrion.

2. the shape of mitochondrion


It is grainy or bacilliform or linear .
its diameter is 0.5~1m , its length is 1.5~3.0mthere are huge mitochondrion in cell of pancreas, its length is 10~20m . There are about 1000--2000 mitochondrion in the cell of liver. The ripe red cell of many mammalians have no mitochondrion. it generally distribute hearty region of the cell .

3.The chemical composition of mitochondrion mitochondrion consist of lipid and protein. protein constitute about 65% -70% of mitochondrion, lipid constitute about 25% -30% of mitochondrion. most of proteins are enzymes.

4.The ultrastructure of mitochondrion


What is ultrastructure? Mitochondrion is close cystiform structure that two unit film fold it .it consist of four parts:
The organelle is composed of compartments that carry out specialized functions. These compartments or regions include the outer membrane, the intermembrane space, the inner membrane, and the cristae and matrix.

outer membrane inner membrane Intermembrane space cristae matrix

outer membrane inner membrane Intermembrane space cristae matrix

A mitochondrion contains outer and inner membranes composed of phospholipid bilayers and proteins. The two membranes, however, have different properties. Because of this double-membraned organization, there are five distinct compartments within the mitochondrion. There is the outer membrane, the intermembrane space (the space between the outer and inner membranes), the inner membrane, the cristae space (formed by infoldings of the inner membrane), and the matrix (space within the inner membrane).

Outer menbrane
It is fairly smooth. It is composed of phospholipid bilayer protein. it have channels protein:hole protein permit that small molecule substance freely pass. marker enzyme: monoamine oxidase

Inner membrane The inner mitochondrial membrane contains proteins with four types of functions 1.Those that perform the redox reactions of oxidative phosphorylation 2.ATP synthase, which generates ATP in the matrix 3.Specific transport proteins that regulate metabolite passage into and out of the matrix 4.Protein import machinery.

There is no hole protein in the inner membrane, so penetrability is weak. There is a electron transport chain across the inner membrane, where the most function be finished, such as oxidative phosphorylation.
It forms cristae , which enlarges the area of inner membrane. There are elementary particle (ATP synthase) in the inner membrane. Marker enzyme: cytochrome oxide enzyme.

respiratory chain (electron transport chain )


it is the electron transport chain, it is composed of four compound. its function: transfer hydrogen transfer electron

Four components of respiratory chain


Complex I: NADH dehydrogenase Complex : succinate dehydrogenase Complex : Cytochrome oxidoreductase(bc1)

Complex : cytochrome c-oxidase

The Electron Transport Chain


Four large, multi-subunit protein complexes - complex I is a NADHdehydrogenase -complex II is succinate dehydrogenase - complex III is the cytochrome oxidoreductase (bc1) - complex IV is cytochrome c oxidase

Complex V: ATP synthase


Structure of elementary particle (ATP synthase)
Head section: coupling factor1(F1), it can synthysize ATP. Stalk section: it can regulate the passage of proton.
F1

ATPase OSCP

F0

HP

Basal section: it can form passage of proton, it allow proton to pass.

Cristae
The inner mitochondrial membrane is compartmentalized into numerous cristae, which expand the surface area of the inner mitochondrial membrane, enhancing its ability to produce ATP. These are not simple random folds but rather invaginations of the inner membrane, which can affect overall chemiosmotic function.

intermenbrane space

it contain enzymes. It can catalyze ATP to


create ADP. Marker enzyme: adenylate kinase.

Matrix
The matrix is the space enclosed by the inner membrane. The matrix is important in the production of ATP with the aid of the ATP synthase contained in the inner membrane. The matrix contains a highly-concentrated mixture of hundreds of enzymes, special mitochondrial ribosomes, tRNA, and several copies of the mitochondrial DNA genome. Of the enzymes, the major functions include oxidation of pyruvate and fatty acids, and the citric acid cycle. A published human mitochondrial DNA sequence revealed 16,569 base pairs encoding 37 total genes: 22 tRNA, 2 rRNA, and 13 peptide genes. The 13 mitochondrial peptides in humans are integrated into the inner membrane, along with proteins encoded by genes that reside in the host cell's nucleus.

The function of Mitochondria


Cellular respiration, also known as Cellular oxidation', is one of the key ways a cell gains useful energy. It is the set of the metabolic reactions and processes that take place in organisms' cells to convert biochemical energy from nutrients into ATP, and then release waste products. The reactions involved in respiration are catabolic reactions that involve the oxidation of one molecule and the reduction of another.

1.Celluar oxidation 1) glycolysis ( Glucose 2 pyruvate ) 2) acetyl CoA 3) tricarboxylic acid (TCA) cycle or Krebs

cycle (citric acid cycle)


4)electron transport and oxidative

phosphorylation

Celluar oxidation

2. In 1961Mitchell, Chemiosmotic hypothesis

There are two types of these: nicotinamide adenine

dinucleotide (NAD+) and flavin adenine dinucleotide


(FAD+). The third molecule, of course, is oxygen. Acetyl Co-A enters the Kreb's cycle. It combine with oxaloacetate. they can form citric acid, it is the first reaction that produce citric acid. Citric acid is then broken down and modified in a stepwise fashion , as this happens, hydrogen ions and carbon molecules are released.

2.Chemiosmotic hypothesis
The Mechanism of Oxidative Phosphorylation

The Chemiosmotic Hypothesis is the proposal in 1961, by Peter D. Mitchell, mitochondrion use the energy of NADH and FADH2 to create a proton gradient across the mitochondrial membrane and that this energy was used by a reversible proton pump , the ATP synthase , to create ATP .
1978, Peter Mitchell was awarded the Nobel Prize in Chemistry.

the content Chemiosmotic hypothesis 1) NADH and FADH2 carry the hydrogen ions to the electron transport chain. 2)In this transfering process,it can produce energy,the energy can pump hydrogen ions from matix to intermembrane space. 3) The proton-motive force, hydrogen ions are accumulated in a high concentration,it moves down concentration from intermembrane space to matix . 4)This enter is coupled to ATP synthesis from ADP and phosphate. 5)At last, hydrogen ions combine with the oxygen to make water.

The semi-autonomous of Mitochondria


Genome The human mitochondrial genome is a circular DNA molecule of about 16 kilobases. It encodes 37 genes: 13 for subunits of respiratory complexes I, III, IV and V, 22 for mitochondrial tRNA (for the 20 standard amino acids, plus an extra gene for leucine and serine), and 2 for rRNA. One mitochondrion can contain two to ten copies of its DNA. As in prokaryotes, there is a very high proportion of coding DNA and an absence of repeats. Mitochondrial genes are in general, mitochondrial DNA lacks introns.

mtDNA

Mitochondrial Inheritance and Disease Mitochondrial Inheritance The genome of human mitochondria contains 16,569 base pairs of DNA organized in a closed circle. These encode: 2 ribosomal RNA (rRNA) molecules 22 transfer RNA (tRNA) molecules 13 polypeptides

Mitochondria have their own DNA and Ribosomes Mitochondria have some of their own DNA, ribosomes, and can make many of their own proteins. The DNA is circular and lies in the matrix in structures called "nucleoids". Each nucleoid may contain 4-5 copies of the mitochondrial DNA (mtDNA).

mitochondrial DNA

Mitochondria also have their own ribosomes and tRNA:


22 tRNAs rRNAs (16S and 12S)

The ribosomes can actually be visualized in some mitochondria. In these figures, they are seen in the matrix as small dark bodies. DNA can also be visualized in mitochondria. The DNA is circular and resembles that of a bacterium in its basic structure.

Summary

The ultrastructure of mitochondrion


cristae, matrix electron transport chain (Four components of respiratory chain) elementary particle (ATP synthase)

outer membrane, inner membrane, Intermembrane space


The function of mitochondrion


Chemiosmotic hypothesis

Celluar oxidation

The semi-autonomous of Mitochondria

mtDNA

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