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What are mitochondria?
Organelles that produce ATP and are called the powerhouse of the cell
Why are mitochondria called the powerhouse of the cell?
Because they synthesize ATP which provides usable energy for the cell
What is ATP?
The usable energy currency of the cell
What is the main function of mitochondria?
To produce ATP for cellular energy
What are the main structural parts of a mitochondrion?
Outer membrane inner membrane intermembrane space cristae and matrix
What is the outer mitochondrial membrane?
The membrane surrounding the outside of the mitochondrion
What is the inner mitochondrial membrane?
The inner membrane that contains components of the electron transport chain
What is the intermembrane space?
The space between the outer and inner mitochondrial membranes
What are cristae?
Folds of the inner mitochondrial membrane
Why are cristae useful?
They increase the surface area of the inner mitochondrial membrane
What is the mitochondrial matrix?
The space enclosed by the inner mitochondrial membrane
What important system is found in the inner mitochondrial membrane?
The electron transport chain
What does ETC stand for?
Electron transport chain
What does the electron transport chain help produce?
ATP
What happens to energy released from food molecules during mitochondrial oxidation?
It is trapped in the form of ATP
What food molecules can provide energy for ATP production?
Glucose fatty acids and amino acids
What is oxidative phosphorylation?
The process in which energy released from oxidation is captured in the form of ATP
What is the basic energy pathway involving mitochondria?
Food molecules → oxidation → electron transport chain → ATP
What metabolic pathways occur in the mitochondrial matrix according to the slides?
TCA cycle fatty acid oxidation and ketone body formation
Where does the TCA cycle occur?
In the mitochondrial matrix
Where does fatty acid oxidation occur according to the mitochondrial slide?
In the mitochondrial matrix
Where does ketone body formation occur?
In the mitochondrial matrix
What is the TCA cycle?
A metabolic pathway that takes place in the mitochondrial matrix
What are ketone bodies?
Molecules formed during fat metabolism that can be used as an alternative energy source
Do mitochondria contain their own DNA?
Yes
What shape is mitochondrial DNA?
Circular
What does mitochondrial DNA produce?
Some mitochondrial membrane proteins
Do mitochondria contain ribosomes?
Yes
What do ribosomes do?
They make proteins
From which parent is mitochondrial DNA inherited?
The mother
What type of inheritance do mitochondrial diseases show according to the slides?
Maternal inheritance
What does maternal inheritance mean?
Inheritance from the mother
What is the connection between mitochondrial DNA and maternal inheritance?
Mitochondrial DNA is inherited from the mother so mitochondrial DNA diseases can show maternal inheritance
What is cytochrome C?
A mitochondrial protein that can initiate programmed cell death
What process can cytochrome C initiate?
Apoptosis
What is apoptosis?
Programmed cell death
What is the relationship between mitochondria and apoptosis?
Cytochrome C from mitochondria can initiate apoptosis
What ion can mitochondria temporarily store?
Calcium
How do mitochondria contribute to calcium homeostasis?
They can temporarily store calcium
What is homeostasis?
Maintaining stable internal conditions
What is calcium homeostasis?
Maintaining balanced calcium levels in the cell
What can happen when genetic disorders affect mitochondria?
Mitochondrial enzymes can have abnormal structure or function and oxidative phosphorylation may not happen properly
Why can mitochondrial genetic disorders cause disease?
Because abnormal mitochondrial enzymes can interfere with oxidative phosphorylation
What is muscle atrophy?
Loss or shrinking of muscle tissue
What muscular problem can mitochondrial disorders cause?
Muscle atrophy
What heart problem can mitochondrial disorders cause?
Abnormal heartbeats
What does CNS stand for?
Central nervous system
What structures make up the CNS?
Brain and spinal cord
What nervous system problem can mitochondrial disorders cause?
CNS degeneration
What does CNS degeneration mean?
Deterioration or damage of the central nervous system
What does renal mean?
Related to the kidneys
What kidney problem can mitochondrial disorders cause?
Renal damage
What symptoms are listed for mitochondrial disorders?
Muscle atrophy abnormal heartbeats CNS degeneration and renal damage
What are lysosomes?
Membrane-bound vesicles containing digestive enzymes
What is a vesicle?
A small membrane-bound sac
What is the main function of lysosomes?
To digest and degrade cellular material and other substances
What type of enzymes do lysosomes contain?
Hydrolytic enzymes called hydrolases
What are hydrolases?
Enzymes that digest or degrade molecules
What can lysosomal hydrolases digest?
Proteins carbohydrates and nucleic acids
Where are lysosomal hydrolases made?
In the endoplasmic reticulum
What happens to lysosomal hydrolases in the Golgi apparatus?
They receive an M6P tag
What does M6P stand for?
Mannose-6-phosphate
What is the purpose of the M6P tag?
It directs hydrolases to lysosomes
What is the pathway of lysosomal hydrolases?
ER → Golgi → M6P tag → lysosome
What does the ER do in the lysosomal enzyme pathway?
It makes the hydrolases
What does the Golgi do in the lysosomal enzyme pathway?
It gives the hydrolases an M6P tag
Where do M6P-tagged hydrolases go?
To lysosomes
What is the approximate pH of the cytosol?
7.4
What is the approximate pH inside a lysosome?
4.8
Is the inside of a lysosome acidic or basic?
Acidic
Why is the acidic environment of lysosomes important?
Lysosomal hydrolases require an acidic pH to work
What is cytosol?
The fluid portion inside the cell
What types of cellular material can enter lysosomes for breakdown?
Cell debris damaged organelles and cytoplasmic material
What is autophagy?
The process in which a cell digests and recycles its own components
What does auto mean in autophagy?
Self
What does autophagy help remove?
Cell debris damaged organelles and cytoplasmic material
What happens to cell debris and damaged organelles during autophagy?
They enter lysosomes and are broken down
What happens during prolonged starvation?
The cell can digest its own components through autophagy to help generate energy
How can autophagy help a cell survive prolonged starvation?
The cell breaks down its own components to obtain useful materials and energy
What can happen to products produced by lysosomal breakdown?
They can return to the cytosol for reuse
Why can lysosomes be described as recycling organelles?
They break down cellular material and allow breakdown products to be reused
Can lysosomes digest material from outside the cell?
Yes
What external material can lysosomes digest?
Foreign particles brought into the cell
What happens if lysosomal hydrolytic enzymes are defective?
Materials cannot be broken down properly and can accumulate inside cells and tissues
What are lysosomal storage diseases?
Inherited metabolic disorders caused by defects in lysosomal hydrolytic enzymes
Why are they called lysosomal storage diseases?
Because substances that should be degraded accumulate or become stored in cells
What substances can accumulate in lysosomal storage diseases?
Lipids and carbohydrates
What can excessive storage of substances eventually cause?
Permanent cellular and tissue damage
Which tissues and organs can be particularly affected by lysosomal storage diseases?
Brain peripheral nervous system liver spleen and bone marrow
What is the peripheral nervous system?
The nerves outside the brain and spinal cord
What is bone marrow?
Soft tissue inside bones where blood cells are produced
What is the basic pathway of a lysosomal storage disease?
Defective hydrolase → poor breakdown → substance accumulation → cellular and tissue damage
What are peroxisomes?
Organelles involved in oxidation of very-long-chain fatty acids plasmalogen synthesis and hydrogen peroxide handling
What type of fatty acids are oxidized by peroxisomes?
Very-long-chain fatty acids
What does VLCFA stand for?
Very-long-chain fatty acid
What do peroxisomes do to very-long-chain fatty acids?
They carry out their oxidation
What important phospholipid do peroxisomes synthesize?
Plasmalogen
What are plasmalogens?
Important phospholipids essential for myelin formation and brain function
Why are plasmalogens important?
They are essential for myelin formation and brain function
What is myelin?
An insulating covering around many nerve fibers