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Part 7 Lecture Mitochondria

Mitochondria are bean-shaped organelles that generate ATP through oxidative phosphorylation. They contain two membranes - an outer and inner membrane. The inner membrane folds inward to form cristae where ATP synthase is located. Mitochondria break down nutrients through glycolysis in the cytosol and the Krebs cycle in the matrix. This generates NADH and FADH2, whose electrons are transferred through electron transport chain complexes embedded in the inner membrane. This pumps protons out of the matrix, creating a proton gradient used by ATP synthase to generate ATP through chemiosmosis. Mitochondrial mutations have been linked to diseases like Parkinson's.

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Eric Jones Uy
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151 views13 pages

Part 7 Lecture Mitochondria

Mitochondria are bean-shaped organelles that generate ATP through oxidative phosphorylation. They contain two membranes - an outer and inner membrane. The inner membrane folds inward to form cristae where ATP synthase is located. Mitochondria break down nutrients through glycolysis in the cytosol and the Krebs cycle in the matrix. This generates NADH and FADH2, whose electrons are transferred through electron transport chain complexes embedded in the inner membrane. This pumps protons out of the matrix, creating a proton gradient used by ATP synthase to generate ATP through chemiosmosis. Mitochondrial mutations have been linked to diseases like Parkinson's.

Uploaded by

Eric Jones Uy
Copyright
© Attribution Non-Commercial (BY-NC)
We take content rights seriously. If you suspect this is your content, claim it here.
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MITOCHONDRIA:

STRUCTURE AND FUNCTION


Mitochondria
 Individual, bean-shaped organelles ranging from 1-
4 µm in length
 May also appear as highly branched,
interconnected tubular network
 Have the ability to fuse with one another (fusion),
or split in 2 (fission)
 Functions:
1. Generates ATP
2. Synthesis of Heme groups and certain amino acid
++
3. Plays a vital role in the uptake and release of Ca

4. Regulates apoptosis
Mitochondrial membranes
1. Outer mitochondrial membrane
 Encloses the mitochondrion, serving its outer
boundary
 Composed of approximately 50% lipid by weight
 Contains a mixture of enzymes involved in diverse
activities like oxidation of epinephrine,
degradation of tryptophan, and elongation of fatty
acids
 Contains “porins”

2. Inner mitochondrial membrane


 Has very high protein/ lipid ratio (>3 : 1)
 Devoid of cholesterol
 Rich in cardiolipin which facilitates the activity of
the proteins involved in ATP synthesis
 Highly impermeable
 Subdivided into 2 interconnected domains:
a. Inner boundary membrane
>lies just inside the outer membrane, forming a
double membrane outer envelope
>rich in CHONs responsible for import of
mitochondrial CHONs

b. Internal cristal membrane

Cristae
 A series of invaginated sheets of deep folds
present within the inner mitochondrial membrane
 Responsible for the role of mitochondria as energy
transducers (ATP synthase)
Cristae junctions
A narrow junctions which joins the 2
interconnected domains of the inner
mitochondrial membrane

Mitochondrial compartments:
1. Intermembrane space
 Region between the outer and inner membrane
 Contains CHONs which initiates apoptosis

2. Matrix
 Region inside the inner membrane
 Has a gel like consistency owing to the presence

of high concentration of H2O soluble CHONs


 Contains ribosomes and DNA

Energy Transducing Mechanism


I. Glycolysis
 Occurs in the cytosol
Net Reaction:
Glucose + 2NAD+ + 2ADP + 2 Pi
→ 2 Pyruvate + 2 ATP + 2 NADH + 2H+ + 2H2O

Fates of Pyruvate:

Fates of NADH:
The electrons of NADH are used to reduce a low-
molecular-weight metabolite that can either:
1. Enter the mitochondrion by a pathway called

malate-aspartate shuttle) and reduce NAD+ to


NADH
2. Transfer its electron to FAD to FADH2 by a

pathway called the glycerol phosphate shuttle


II. Kreb’s cycle/ Tricarboxylic acid cycle/ Citirc Acid
cycle
> occurs in the mitochondrial matrix
Net Equation:
2 Acetyl CoA + 4H2O + 2FAD + 6 NAD+ + 2GDP + 2Pi
→ 4CO2 + 2FADH2 + 6NADH + 6H+ + 2GTP + 2 HS-CoA

III. Electron Trasport Chain (Respiratory Chain)


 Location: inner mitochondrial membrane
 Coupled with proton translocation from the matrix
to the intermembrane space

Electron carriers:
 With the exception of ubiquinone, all are
prosthetic groups (non-amino acid components
that are tightly associated)
 Types:
1. Flavoproteins
 With attached FAD (flavin adenine dinucleotide) or
FMN (flavin mononucleotide)
 Capable of accepting and donating two protons
and 2 electrons

2. Cytochromes
 Contain heme prosthetic groups
 3 distinct types : a, b & c
 Able to accept and donate 1 electron

3. 3 copper atoms
 Accept and donate a single electron as they

alternate between the Cu++ and Cu+ states

4. Ubiquinone (UQ or coenzyme Q)


 Lipid-soluble molecule containing isoprenoid units
 Able to accept and donate 2 electrons and 2
protons

5. Iron-sulfur proteins
 Iron are linked to inorganic sulfide ions as part of
iron-sulfur center
 Able to accept and donate only a single electron

Electron Transport Complexes:


1. Complex I (NADH dehydrogenase)
 Transfer electrons from NADH to FMN to Fe-S and
finally to ubiquinone embedded in the lipid bilayer

2.Complex III (cytochrome bc1)


 Transfer electrons from:
a. UQ to Fe-S to cyt c1
b. UQ to cyt b

3. Complex II (succinate dehydrogenase)


 Feeds lower-energy electrons from succinate to
FAS and then to UQ
 Not accompanied by proton translocation

4. Complex IV (cytochrome c oxidase)


 Transfer electrons from reduced cytochrome to

oxygen forming H2O

To reduce a whole molecule of O2:


4 cyt c2+ + 4 H+ + O2 → 4 cyt c + 2 H2O

For every molecule of O2 reduced to 2 H2O by


cytochrome oxidase, 4 H+ are being translocated.
IV. Translocation of Protons and establishment of a
Proton Motive Force

Proton motive force


 Has 2 components: 1. Voltage (80%)
2. pH gradient (20%)
 Maintenance requires that the inner mitochondrial

membrane remains impermeable


 Used to drive ATP synthesis
 Other functions:
1. Uptake of ADP into the mitochondtion in
exchange for the release of ATP to the cytosol
2. Uptake of phosphate and Calcium ions
3. Import of mitochondrial proteins

2,4 dinitrophenol (DNP)


 A certain lipid-soluble agents that enables
continuation of oxidation of substrates without
being able to generate ATP
 Uncouples glucose oxidation and ADP
phosphorylation by combining with protons and
carrying them across the inner mitochondrial
membrane down their electrochemical gradient

Uncoupling proteins (UCPs)


 Acts as natural/endogenous uncouplers
 Abundant in brown adipose tissue of mammals,
which functions as a source of heat production
during exposure to cold temperatures
V. Oxidative phosphorylation
Steps:
1. Substrates are oxidized and the electrons are

transferred to coenzymes forming NADH or FADH2.


These high energy electrons are then transferred
through a series of electron carrieirs of the ETS.
The energy released is used to traslocate protons
from the matrix to the intermembrane space,
establishing a proton electrochemical gradient
across the inner mitochondrial membrane.
2. The protons move down the electrochemical
gradient, through an ATP-synthesizing complex.
The energy stored in the gradient is used to
synthesize ATP
ATP synthase
 The ATP synthesizing enzyme
 Contains 2 distinct parts:
1. F1 head
> projects into the matrix
> contains the catalytic site
> consist of 5 different polypeptides (α3β3δγε)

2. F0 base
> Embedded in the lipid bilayer and forms a
channel through which protons are conducted
from the intermembrane space into the matrix
> Consist of 3 different polypeptides

Binding Change Mehanism:


1. Movement of protons induces rotation of the γ

subunit, which will change the conformation of F1


catalytic sites
2. The energy released by the movement of protons
is not used to drive ADP phosphorylation directly
but principally to change the binding affinity of the
active site for the ATP product
3. Each active site progresse successively through 3
distinct conformation that have different affinities
for substrates and product
a. “L” or “loose” conformation (ADP and Pi are
loosely bound)
b. “T” or “tight” conformation (ADP and Pi or ATP
are tightly bound
c. “O” or open conformation (has very low affinity
for nucleotides, allowing release of ATP)
Mitochondrial mutations
 Likely to accumulate in cells that remain in the
body for long periods of time (e.g., nerve and
muscle tissues)
 Has been implicated in premature aging

Parkinson’s Disease
 Neurodegenerative disease associated with
mitochondrial mutation leading to decrease in
Complex I activity

Aerobic vs. anaerobic exercise


Anaerobic Aerobic
1. Example Lifting weights, sprints Bicycling, fast walking
2. Types of muscle Fast twitch fibers Slow twitch fibers
fiber
3. Generation of ATP Glycolysis Aerobic respiration
4. Substrate utilized Glycogen stores Initially glycogen but
eventually fatty acid
from adipose tissues
5. Lactic acid Yes No
accumulation
6. Sustainability Short Longer

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