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MITOCHONDRIA
STRUCTURE AND FUNCTION
Mitochondria Overview
• Mitochondria are always referred to as the
powerhouse of the cell.
• Mitochondria has sausage-shaped kind of like
organelles.
Mitochondria Double-layered
membrane
1. Outer Membrane
Made of a phospholipid bilayer
The outer membrane is very permeable
Allows molecules to move in and out
• 2. Inner Membrane
Another phospholipid bilayer
Not very permeable
 has lots of protein transporters and they very
closely regulate what is going inside and
outside of the mitochondria
Intermembrane space
• Space that exists between outer and inner
membrane
• Highly concentrated with proton ions (H+)
i. Helpful in electron transport chain
Cristae
• Within the inner membrane, there’s a specific
type of invaginations
i. Continuation of the inner membrane but
invaginates into the center
• The purpose is to increase the surface area for
lots of specific types of reaction
MITOCHONDRIAL MATRIX
• There are different solutes in it
• There are two particular things in the matrix
1. Mitochondrial DNA
2. Ribosomes
Mitochondrial DNA
• This is maternal DNA (only inherited from
mother)
• Has genes to make particular RNA to make
proteins
RIBOSOMES
• We need ribosomes to be able to translate
that RNA and make proteins
• These are proteins the mitochondria can make
on their own to help with their function
Functions of Mitochondria
1. Outer and Inner Membrane
• Outer and inner membrane plays a important
role in protein transport.
Protein transport
• Mitochondria have DNA
• Mitochondrial DNA can make RNA, which can
help to make some proteins
• But it doesn’t make all the proteins that it
needs to be able to function ▪ So, we have to
take some proteins from the nucleus
• The nucleus will help us to make RNA
• RNA will then make proteins
• And these proteins have to get sent into the
mitochondria
• Nucleus DNA makes RNA → come out via
nuclear pore
• mRNA will then combine with ribosomes
• Ribosomes will then synthesize proteins
• These proteins are unfolded. They’re not in
active form
Translocase of the outer membrane
• Mitochondria has special transporters that are specialized
in moving these unfolded proteins that it needs to be able
to perform reactions inside of the mitochondria
• It needs to transport across
• These proteins will get chaperoned, and then they will
bind to the little receptor
• The transporter is called translocase of the outer
membrane (TOM)
• Once the unfolded protein binds to the receptor It will
open up TOM and move this unfolded protein into the
intermembrane space
Translocase of the inner membrane
(TIM)
• Little chaperone protein will bind to it and
bring it to the next receptor on the inner
membrane
• It stimulates the receptor
• It opens up the next channel translocase of
the inner membrane (TIM) and pushes the
unfolded protein into the mitochondrial
matrix
In the mitochondrial matrix
• These proteins may be needed to perform
specific things, Maybe it’s an enzyme that
plays a role in lots of metabolic reactions
• So, we have some specific types of proteases
or other enzymes that will work on these
proteins and properly fold him together and
activate.
• and make into a specific special enzyme
In short
• Mitochondrial DNA only makes 15% of the
protein that’s needed That means we need
lots of proteins from the nucleus to help us to
perform a lot of the functions
• Mitochondria accepts protein in unfolded
form, brings them, and activates them by
folding them properly and then uses those for
their metabolic reactions
Functions of Mitochondria
2. Miscellaneous transport
Miscellaneous transport
● For metabolic reactions to occur, we have to
move things right across the actual mitochondrial
membrane in and out
1) Carbohydrates
● Glycolysis, or gluconeogenesis has to occur
inside the mitochondrial matrix
2) Fatty acids
● Because they play a role in beta-oxidation
3) Amino acids
● Because they play a role in the urea cycle
ELECTRON TRANSPORT CHAIN
• ETC is Very critical to the function of the mitochondria
• Mitochondria is often time called as powerhouse of the
cell o Produces ATP
• ATP is power energy
• Found primarily on the inner membrane. We don’t see this
involved in the outer membrane
• these protein molecules make up these different
complexes like complex I-IV and a very special molecule
called ATP synthase
• (1) Conversion of pyruvate to acetyl-CoA
• Pyruvate gets converted into acetyl-CoA
• (2) Krebs cycle
• Acetyl-CoA goes to Krebs cycle
• From the Krebs cycle that occurs in the
mitochondria We have high-energy electron
transporters called FADH2 and NADH They carry
lots of electrons
• They have hydrogen called hydride ion
(3) Electron transport chain –
Oxidative phosphorylation
• They take, and transport these electrons onto these
proteins
• They drop the electrons off in them, and then these
molecules pass these electrons down the chain from
areas of high energy to areas of low energy
• Each time that happens, it pumps all these protons
(H+) out into the intermembrane space Remember,
intermembrane space is very rich in H+ (protons)
• H+ moves down the ATP synthase powerfully It
creates energy that the protein can harvest
• Naturally, ADP is stuck to ATP synthase
• As the H+ runs down the gradient
• As the electrons are being passed down ▪ It
creates energies that fuse these to form ATP
• This process is called oxidative phosphorylation
Side effect
● A side effect is that All these electrons that are getting
passed along the membrane
● Sometimes, some of these electrons can combine with
O2 and other molecules and H2O2 (hydrogen peroxide)
Superoxide free radicals (e.g., O2-) are formed.
● So basically, they can increase the formation of
reactive oxygen species (ROS) , Which is that unfortunate
side reaction from the electron transport chain
REACTIONS THAT OCCUR IN THE
MITOCHONDRIAL MATRIX
● There are lots of metabolic reactions that can
occur in the mitochondria
• Especially, inner and outer membrane,
mitochondrial matrix, and cytoplasm
(1) Conversion of pyruvate into acetyl-CoA
● Once the glucose gets into the cell, it will
eventually get converted into pyruvate via
glycolysis
● Pyruvate will be into the mitochondrial
matrix, It undergoes a conversion into acetyl-
CoA
(2) Krebs cycle
● Acetyl-CoA will go into the cycle called the Krebs cycle
● Coming off of the Krebs cycle is high energy molecules
that carry electrons NADH and FADH2
● These go to electron transport chains, where they pass
off those electrons Pump H+ into the intermembrane
space
They help to generate ATP via oxidative phosphorylation
(3) 𝒃𝒆𝒕𝒂−𝒃𝒆𝒕𝒂−𝒐𝒙𝒊𝒅𝒂𝒕𝒊𝒐𝒏
● fatty acids A long chain, sometimes it can be 16
carbons long
• We can bring these into the mitochondrial matrix
across the membrane
• They can go through a series of metabolic
reactions where they get broken down into
acetyl-CoA to make energy
• This step is called 𝜷- oxidation
(4) Urea cycle
• Amino acids can be brought into the mitochondria as well
• Eventually, they can get metabolized and give way to products in
the Krebs cycle ,When it does that, it can give off ammonia
• Ammonia is really toxic
• Ammonia will go through a series of reactions called the urea
cycle
• It liberates urea which is less toxic in comparison to ammonia, and
it’s easier to be excreted
• The urea cycle occurs specifically in the mitochondrion and a little
bit in the cytoplasm
• (5) Gluconeogenesis
• We can take amino acids or odd-chain fatty acids,
and we can convert them into a specific substrate
• We can convert them back into pyruvate and
then back into glucose
• What is it called when we take molecules like
amino acid and odd chain fatty acids and make
glucose? Gluconeogenesis
• (6) From the Krebs cycle, we can take some of
these intermediates and make molecule
called heme
• Some of these molecules are synthesized in 2
places
• Mitochondrial matrix
• Cytoplasm
7) Ketogenesis
• We can take acetyl-CoA, and we can shunt
them into making molecules called ketones
• Occurs in the mitochondrial matrix
• This process is called ketogenesis
APOPTOSIS
• Whenever there’s some process where a cell is infected or
is cancerous, or it needs to die o There are special
molecules present inside of mitochondrial matrix called
cytochrome C
• Normally, these molecules do not leak out
• So, there’s a protein called bcl2 that helps to prevent this
from occurring
• In apoptosis, we decrease the number of bcl2
• Now, we can’t control the cytochrome C molecules from
leaking out
• Cytochrome C molecules activate enzymes called
caspases
• Caspases are basically proteases
• They start ripping through a bunch of different
cells
• Damaging cell membranes, organelles
• And eventually cause the cell to undergo the
death process
Any questions

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MITOCHONDRIA Is The powerhouse of the cell.pptx

  • 3. • Mitochondria are always referred to as the powerhouse of the cell. • Mitochondria has sausage-shaped kind of like organelles.
  • 4. Mitochondria Double-layered membrane 1. Outer Membrane Made of a phospholipid bilayer The outer membrane is very permeable Allows molecules to move in and out
  • 5. • 2. Inner Membrane Another phospholipid bilayer Not very permeable  has lots of protein transporters and they very closely regulate what is going inside and outside of the mitochondria
  • 6. Intermembrane space • Space that exists between outer and inner membrane • Highly concentrated with proton ions (H+) i. Helpful in electron transport chain
  • 7. Cristae • Within the inner membrane, there’s a specific type of invaginations i. Continuation of the inner membrane but invaginates into the center • The purpose is to increase the surface area for lots of specific types of reaction
  • 8. MITOCHONDRIAL MATRIX • There are different solutes in it • There are two particular things in the matrix 1. Mitochondrial DNA 2. Ribosomes
  • 9. Mitochondrial DNA • This is maternal DNA (only inherited from mother) • Has genes to make particular RNA to make proteins
  • 10. RIBOSOMES • We need ribosomes to be able to translate that RNA and make proteins • These are proteins the mitochondria can make on their own to help with their function
  • 11. Functions of Mitochondria 1. Outer and Inner Membrane
  • 12. • Outer and inner membrane plays a important role in protein transport.
  • 14. • Mitochondria have DNA • Mitochondrial DNA can make RNA, which can help to make some proteins • But it doesn’t make all the proteins that it needs to be able to function ▪ So, we have to take some proteins from the nucleus
  • 15. • The nucleus will help us to make RNA • RNA will then make proteins • And these proteins have to get sent into the mitochondria
  • 16. • Nucleus DNA makes RNA → come out via nuclear pore • mRNA will then combine with ribosomes • Ribosomes will then synthesize proteins • These proteins are unfolded. They’re not in active form
  • 17. Translocase of the outer membrane • Mitochondria has special transporters that are specialized in moving these unfolded proteins that it needs to be able to perform reactions inside of the mitochondria • It needs to transport across • These proteins will get chaperoned, and then they will bind to the little receptor • The transporter is called translocase of the outer membrane (TOM) • Once the unfolded protein binds to the receptor It will open up TOM and move this unfolded protein into the intermembrane space
  • 18. Translocase of the inner membrane (TIM) • Little chaperone protein will bind to it and bring it to the next receptor on the inner membrane • It stimulates the receptor • It opens up the next channel translocase of the inner membrane (TIM) and pushes the unfolded protein into the mitochondrial matrix
  • 19. In the mitochondrial matrix • These proteins may be needed to perform specific things, Maybe it’s an enzyme that plays a role in lots of metabolic reactions • So, we have some specific types of proteases or other enzymes that will work on these proteins and properly fold him together and activate. • and make into a specific special enzyme
  • 20. In short • Mitochondrial DNA only makes 15% of the protein that’s needed That means we need lots of proteins from the nucleus to help us to perform a lot of the functions • Mitochondria accepts protein in unfolded form, brings them, and activates them by folding them properly and then uses those for their metabolic reactions
  • 21. Functions of Mitochondria 2. Miscellaneous transport
  • 23. ● For metabolic reactions to occur, we have to move things right across the actual mitochondrial membrane in and out 1) Carbohydrates ● Glycolysis, or gluconeogenesis has to occur inside the mitochondrial matrix 2) Fatty acids ● Because they play a role in beta-oxidation 3) Amino acids ● Because they play a role in the urea cycle
  • 25.
  • 26. • ETC is Very critical to the function of the mitochondria • Mitochondria is often time called as powerhouse of the cell o Produces ATP • ATP is power energy • Found primarily on the inner membrane. We don’t see this involved in the outer membrane • these protein molecules make up these different complexes like complex I-IV and a very special molecule called ATP synthase
  • 27. • (1) Conversion of pyruvate to acetyl-CoA • Pyruvate gets converted into acetyl-CoA • (2) Krebs cycle • Acetyl-CoA goes to Krebs cycle • From the Krebs cycle that occurs in the mitochondria We have high-energy electron transporters called FADH2 and NADH They carry lots of electrons • They have hydrogen called hydride ion
  • 28. (3) Electron transport chain – Oxidative phosphorylation • They take, and transport these electrons onto these proteins • They drop the electrons off in them, and then these molecules pass these electrons down the chain from areas of high energy to areas of low energy • Each time that happens, it pumps all these protons (H+) out into the intermembrane space Remember, intermembrane space is very rich in H+ (protons)
  • 29. • H+ moves down the ATP synthase powerfully It creates energy that the protein can harvest • Naturally, ADP is stuck to ATP synthase • As the H+ runs down the gradient • As the electrons are being passed down ▪ It creates energies that fuse these to form ATP • This process is called oxidative phosphorylation
  • 30. Side effect ● A side effect is that All these electrons that are getting passed along the membrane ● Sometimes, some of these electrons can combine with O2 and other molecules and H2O2 (hydrogen peroxide) Superoxide free radicals (e.g., O2-) are formed. ● So basically, they can increase the formation of reactive oxygen species (ROS) , Which is that unfortunate side reaction from the electron transport chain
  • 31. REACTIONS THAT OCCUR IN THE MITOCHONDRIAL MATRIX
  • 32.
  • 33. ● There are lots of metabolic reactions that can occur in the mitochondria • Especially, inner and outer membrane, mitochondrial matrix, and cytoplasm
  • 34. (1) Conversion of pyruvate into acetyl-CoA ● Once the glucose gets into the cell, it will eventually get converted into pyruvate via glycolysis ● Pyruvate will be into the mitochondrial matrix, It undergoes a conversion into acetyl- CoA
  • 35. (2) Krebs cycle ● Acetyl-CoA will go into the cycle called the Krebs cycle ● Coming off of the Krebs cycle is high energy molecules that carry electrons NADH and FADH2 ● These go to electron transport chains, where they pass off those electrons Pump H+ into the intermembrane space They help to generate ATP via oxidative phosphorylation
  • 36. (3) 𝒃𝒆𝒕𝒂−𝒃𝒆𝒕𝒂−𝒐𝒙𝒊𝒅𝒂𝒕𝒊𝒐𝒏 ● fatty acids A long chain, sometimes it can be 16 carbons long • We can bring these into the mitochondrial matrix across the membrane • They can go through a series of metabolic reactions where they get broken down into acetyl-CoA to make energy • This step is called 𝜷- oxidation
  • 37. (4) Urea cycle • Amino acids can be brought into the mitochondria as well • Eventually, they can get metabolized and give way to products in the Krebs cycle ,When it does that, it can give off ammonia • Ammonia is really toxic • Ammonia will go through a series of reactions called the urea cycle • It liberates urea which is less toxic in comparison to ammonia, and it’s easier to be excreted • The urea cycle occurs specifically in the mitochondrion and a little bit in the cytoplasm
  • 38. • (5) Gluconeogenesis • We can take amino acids or odd-chain fatty acids, and we can convert them into a specific substrate • We can convert them back into pyruvate and then back into glucose • What is it called when we take molecules like amino acid and odd chain fatty acids and make glucose? Gluconeogenesis
  • 39. • (6) From the Krebs cycle, we can take some of these intermediates and make molecule called heme • Some of these molecules are synthesized in 2 places • Mitochondrial matrix • Cytoplasm
  • 40. 7) Ketogenesis • We can take acetyl-CoA, and we can shunt them into making molecules called ketones • Occurs in the mitochondrial matrix • This process is called ketogenesis
  • 42. • Whenever there’s some process where a cell is infected or is cancerous, or it needs to die o There are special molecules present inside of mitochondrial matrix called cytochrome C • Normally, these molecules do not leak out • So, there’s a protein called bcl2 that helps to prevent this from occurring • In apoptosis, we decrease the number of bcl2 • Now, we can’t control the cytochrome C molecules from leaking out
  • 43. • Cytochrome C molecules activate enzymes called caspases • Caspases are basically proteases • They start ripping through a bunch of different cells • Damaging cell membranes, organelles • And eventually cause the cell to undergo the death process