Biomedical Sciences I: Mitochondrion Quiz
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Questions and Answers

Which complex does FADH2 enter the electron transport chain?

  • Complex III
  • Complex II (correct)
  • Complex I
  • Complex IV
  • What is produced as a result of electron transfer to O2 in the electron transport chain?

  • Ubiquinone
  • H2O (correct)
  • NADH
  • ATP
  • How many molecules of ATP can be generated from a single molecule of glucose?

  • 36 (correct)
  • 18
  • 30
  • 42
  • What triggers the activation of caspases during apoptosis?

    <p>Cleavage of specific aspartic acids (B)</p> Signup and view all the answers

    What type of proteins do mitochondrially encoded genes typically influence?

    <p>Mitochondrial proteins (D)</p> Signup and view all the answers

    What successfully builds an electrochemical gradient in aerobic metabolism?

    <p>Protons (H+) (C)</p> Signup and view all the answers

    The spherical F1 head of ATP synthase is primarily involved in which reaction?

    <p>Phosphorylation of ADP to ATP (C)</p> Signup and view all the answers

    What characterizes mitochondrial myopathies?

    <p>Neuromuscular symptoms (D)</p> Signup and view all the answers

    What is the primary function of complex III in the electron transport chain?

    <p>Transfers electrons to cytochrome c (B)</p> Signup and view all the answers

    Which pathway is NOT one of the two best-understood activation mechanisms for caspases?

    <p>Autophagic pathway (B)</p> Signup and view all the answers

    What role does the TOM complex play in mitochondrial function?

    <p>Import of nearly all nucleus-encoded mitochondrial proteins (A)</p> Signup and view all the answers

    Which complex is responsible for the insertion and folding of b-barrel proteins?

    <p>SAM complex (A)</p> Signup and view all the answers

    How do mitochondrial precursors remain in an unfolded state in the cytosol?

    <p>Thanks to chaperones of the hsp70 family (B)</p> Signup and view all the answers

    What is the function of the OXA complex in mitochondria?

    <p>Mediates the insertion of mitochondrial genome-encoded membrane proteins (D)</p> Signup and view all the answers

    What initiates mitochondrial fission?

    <p>Intervention of the endoplasmic reticulum (B)</p> Signup and view all the answers

    What are mitochondria capable of doing as dynamic organelles?

    <p>Fusing with each other and splitting (D)</p> Signup and view all the answers

    What does the interaction of mitochondria with the microtubular cytoskeleton determine?

    <p>Their orientation and distribution within the cell (C)</p> Signup and view all the answers

    What color results from a fusion of a green-fluorescent mitochondrion with a red-fluorescent one?

    <p>Yellow (D)</p> Signup and view all the answers

    What is NOT a component of mitochondrial protein transport?

    <p>Golgi apparatus (D)</p> Signup and view all the answers

    What is a function of the TIM complexes in mitochondria?

    <p>Transport of proteins at the inner membrane (C)</p> Signup and view all the answers

    What characteristic of mitochondria allows them to change shape and move within cells?

    <p>Association with the cytoskeleton (D)</p> Signup and view all the answers

    Which feature is NOT part of the mitochondrial structure?

    <p>Chlorophyll-rich matrix (D)</p> Signup and view all the answers

    Which component of mitochondria is responsible for most ATP production?

    <p>Cristae (C)</p> Signup and view all the answers

    What role does mitochondrial DNA (mtDNA) play in cellular functions?

    <p>Storing genetic information for protein synthesis (B)</p> Signup and view all the answers

    How is mitochondrial DNA inherited in humans?

    <p>Uniparentally from the mother (B)</p> Signup and view all the answers

    What is a biological consequence of the high mutation rate of mtDNA?

    <p>Heteroplasmy in offspring (B)</p> Signup and view all the answers

    What is a primary function of the intermembrane space in mitochondria?

    <p>Initiating cell death mechanisms (D)</p> Signup and view all the answers

    Why are sperm mitochondria eliminated from fertilized eggs?

    <p>To ensure maternal inheritance of mtDNA (C)</p> Signup and view all the answers

    What process occurs when nonfunctional mitochondria undergo organized degradation?

    <p>Mytophagy (B)</p> Signup and view all the answers

    Which of the following best describes the matrix of the mitochondrion?

    <p>Gel-like consistency filled with enzymes (D)</p> Signup and view all the answers

    What is the primary reason for the degradation of mitochondrial DNA in sperm cells?

    <p>To allow maternal dominance of mtDNA (D)</p> Signup and view all the answers

    What occurs to the mitochondrial protein import when there is a loss of the electrochemical gradient?

    <p>It becomes inefficient. (A)</p> Signup and view all the answers

    What is produced when pyruvate is decarboxylated and linked to coenzyme A in the mitochondria?

    <p>Acetyl CoA (A)</p> Signup and view all the answers

    Which organelle regulates the calcium concentration of the cytosol?

    <p>Mitochondrion (D)</p> Signup and view all the answers

    Which steps in aerobic metabolism are directly associated with the generation of NADH?

    <p>Glycolysis and the TCA cycle (D)</p> Signup and view all the answers

    What is the role of PINK1 in the context of mitochondria?

    <p>Phosphorylates ubiquitin and Parkin (A)</p> Signup and view all the answers

    How does NADH produced during glycolysis enter the mitochondrion?

    <p>By the malate-aspartate shuttle (D)</p> Signup and view all the answers

    Which component of the electron transport chain is a soluble protein in the intermembrane space?

    <p>Cytochrome c (D)</p> Signup and view all the answers

    What is the main outcome of the electron transport chain in mitochondria?

    <p>Production of ATP (B)</p> Signup and view all the answers

    What is shared between the mitochondrion and the cytoplasm in the context of heme group biosynthesis?

    <p>Biosynthesis process (C)</p> Signup and view all the answers

    Flashcards

    Mitochondrial Dynamics

    Mitochondria are dynamic structures that move within cells, constantly changing shape, dividing, and fusing. They are also closely associated with the cytoskeleton, which determines their distribution within the cell.

    Mitochondrial Morphology

    Depending on the cell type, mitochondria can have different shapes. Some are individual, bean-shaped organelles, typically 1-4 micrometers in length. Others form highly branched, interconnected tubular networks.

    Endosymbiotic Origin of Mitochondria

    Mitochondria are believed to have evolved from an ancient aerobic bacterium that developed a symbiotic relationship with an anaerobic host cell. This process is known as endosymbiosis.

    Mitochondrial Structure

    Mitochondria are surrounded by a double-membrane system, with an outer membrane, inner membrane, and cristae. The space between the two membranes is called the intermembrane space, and the inner compartment enclosed by the inner membrane is called the matrix.

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    Outer Mitochondrial Membrane

    The outer mitochondrial membrane serves as the outer boundary of the mitochondrion. It's smooth and uniform and freely permeable to ions and small molecules. It's composed of various proteins, including translocases (proteins that help import mitochondrial proteins), and is rich in cardiolipin, but lacks cholesterol.

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    Mitochondrial Cristae

    Cristae are series of invaginated membranous sheets within the inner membrane. They contain enzymes and structural proteins involved in aerobic respiration and ATP production.

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    Intermembrane Space

    The intermembrane space contains proteins involved in the initiation of cell death mechanisms. It has the same pH and ionic composition as the cytoplasm.

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    Mitochondrial Matrix

    The matrix is a gel-like substance within the inner membrane. It's rich in soluble proteins (enzymes for the Krebs cycle), contains a small amount of DNA (mtDNA), and has free ribosomes, smaller than those found in the cytoplasm.

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    Mitochondrial DNA (mtDNA)

    Mitochondrial DNA (mtDNA) encodes for essential proteins, rRNA, and tRNA. It has a high mutation rate, leading to heteroplasmy (a mixture of different mtDNA variants within a cell). This makes it valuable for tracing evolutionary history.

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    Maternal Inheritance of mtDNA

    Unlike nuclear DNA, mitochondrial DNA is inherited maternally. This means offspring receive mtDNA solely from their mother. During fertilization, sperm mitochondria are typically degraded and eliminated from the fertilized egg.

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    Mitochondrial Protein Synthesis

    Most proteins destined for the mitochondria are synthesized by ribosomes in the cytosol and then transported into the organelle.

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    Mitochondrial Translocator Complexes

    Specialized protein complexes embedded within the mitochondrial membranes that facilitate the transport of proteins into the organelle.

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    TOM Complex

    A complex located in the outer mitochondrial membrane essential for importing nearly all proteins encoded by the nucleus.

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    SAM Complex

    A complex responsible for inserting beta barrel proteins into the outer mitochondrial membrane, ensuring their proper folding.

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    TIM Complexes

    Protein complexes embedded in the inner mitochondrial membrane that mediate the import of proteins into the mitochondrial matrix and inner membrane.

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    OXA Complex

    A complex that guides the insertion of membrane proteins encoded by the mitochondrial genome into the inner mitochondrial membrane.

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    Mitochondrial Protein Unfolding

    Proteins destined for the mitochondria remain unfolded in the cytosol, preventing aggregation, thanks to chaperones of the Hsp70 family.

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    Mitochondrial Fusion and Fission

    Mitochondria are dynamic organelles that can change their shape by fusing with each other (fusion) or splitting into separate units (fission).

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    Mitochondria and the Cytoskeleton

    The microtubular cytoskeleton is associated with mitochondria, influencing their orientation and distribution within the cell.

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    ER and Mitochondrial Fission

    The endoplasmic reticulum plays a role in initiating mitochondrial fission, starting the constriction process that eventually splits the organelle.

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    What is mitophagy?

    Mitochondria that are no longer functional undergo a process called mitophagy, a specialized form of autophagy, where they are targeted for degradation within the cell.

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    What happens to PINK1 when a mitochondrion becomes dysfunctional?

    When a mitochondrion loses its electrochemical gradient, its protein import mechanism becomes inefficient. This leads to the accumulation of the PINK1 protein kinase on the outer mitochondrial membrane.

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    How is PINK1 involved in mitophagy?

    Activated PINK1 phosphorylates both mitochondrial ubiquitin and the Parkin protein. These phosphorylations trigger the formation of an autophagosome, a structure that engulfs the damaged mitochondrion for degradation.

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    What is the link between PINK1/Parkin and Parkinson's disease?

    Mutations in the PINK1 and Parkin genes are often associated with Parkinson's disease, a neurodegenerative disorder characterized by the accumulation of dysfunctional mitochondria in neurons.

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    What is the main function of mitochondria?

    Mitochondria are the powerhouses of the cell, converting glucose into ATP through oxidative phosphorylation.

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    What role does mitochondria play in the urea cycle?

    Mitochondria are involved in the urea cycle, a process that removes nitrogenous waste from the body. The two critical steps of the urea cycle occur in the mitochondrial matrix of liver cells.

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    Where is heme synthesis carried out?

    Mitochondria are involved in the synthesis of heme groups, essential components of hemoglobin and other proteins. This process happens in both the mitochondrion and the cytoplasm.

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    What role do mitochondria play in membrane synthesis?

    Mitochondria play a key role in membrane biosynthesis. They synthesize lipids such as phosphatidylethanolamine, phosphatidylglycerol, and phosphatidic acid.

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    What role do mitochondria play in apoptosis?

    Mitochondria play a role in apoptosis, a programmed cell death process, by being involved in the caspase-dependent pathway.

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    How do mitochondria regulate calcium and interact with the ER?

    Mitochondria regulate the concentration of calcium ions in the cytosol. They interact closely with the endoplasmic reticulum (ER) to exchange lipids.

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    Electron Transport Chain: Entry Point

    Electrons from either NADH (via complex I) or FADH2 (via complex II) enter the electron transport chain.

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    Electron Transport Chain: Ubiquinone

    Electrons are passed from complex I or II to ubiquinone.

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    Electron Transport Chain: Complex III and Cytochrome c

    Reduced ubiquinone (ubiquinol) transfers electrons to complex III and then to cytochrome c.

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    Electron Transport Chain: Complex IV and Oxygen

    Cytochrome c carries electrons to complex IV (cytochrome oxidase), where they finally reach oxygen, forming water (H2O).

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    Electron Transport Chain: Proton Gradient

    The energy released as electrons move through the chain is used to pump protons from the mitochondrial matrix across the inner membrane, creating a proton gradient.

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    Electron Transport Chain: ATP Synthesis

    The electrochemical gradient drives protons back into the matrix, powering ATP synthase to generate ATP.

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    Caspase: Definition

    A caspase is a protease (enzyme that breaks down proteins) that plays a crucial role in apoptosis (programmed cell death).

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    Caspase: Activation

    Caspases are synthesized as inactive precursors and activated only during apoptosis.

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    Caspase Activation Pathways

    Two main pathways trigger caspase activation: the extrinsic pathway (activated by external signals) and the intrinsic (mitochondrial) pathway (activated by internal signals).

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    Mitochondrial Diseases: Definition

    Mitochondrial diseases are a heterogeneous group of disorders caused by defects in mitochondria, leading to impaired energy production.

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    Study Notes

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    • Use is permitted for private and study purposes only, not for profit or commercial gain.

    Biomedical Sciences I: Cellular Biology I

    • Course information: Biomedical Sciences I, Cellular Biology I
    • Instructor: Lorena Di Pietro
    • Email: [email protected]

    Mitochondrion and Aerobic Respiration

    • Title: Mitochondrion and Aerobic Respiration.

    Mitochondrion

    • Mitochondria are dynamic structures.
    • They move within cells, constantly changing shape, dividing, and fusing.
    • They are associated with the cytoskeleton, which influences their distribution.
    • Morphology varies depending on cell type, (e.g., individual bean-shaped organelles, highly branched, interconnected tubular networks).
    • Dimensions: 1–4 µm in length.

    Mitochondrion: Endosymbiosis (page 6)

    • Mitochondria evolved from an ancient aerobic bacterium via endosymbiosis.
    • Ancestral eukaryote consumed aerobic bacteria that then became mitochondria.
    • A second endosymbiotic event led to photosynthesis bacteria becoming chloroplasts in the early eukaryote.

    Mitochondrion: Organization and Structure (page 7)

    • Double-membrane system (inner and outer membranes + cristae).
    • Intermembrane space
    • Matrix

    Mitochondrion: Outer Mitochondrial Membrane (page 8)

    • Smooth and uniform outer membrane, similar to the outer bacterial membrane.
    • Freely permeable to small ions and molecules (<50kDa) due to nonselective porins.
    • Contains enzymes with varied functions (e.g., oxidation of epinephrine, degradation of tryptophan, elongation of fatty acids).
    • Composed of 50% lipids.

    Mitochondrion: Inner Mitochondrial Membrane (page 9)

    • Enriched in proteins (>100 different proteins, ~80%).
    • Highly impermeable.
    • Contains two domains:
      • Inner boundary membrane, parallel to the outer membrane, with protein import translocases lacking cholesterol but rich in cardiolipin.
      • Cristae, invaginated membranous sheets with 25% lipids containing enzymes and structural proteins for aerobic respiration and ATP production.
      • Cristae junctions connect inner and outer domains.

    Mitochondrion: Intermembrane Space (page 10)

    • Contains proteins involved in cell death initiation.
    • pH and ionic composition similar to cytoplasm.

    Mitochondrion: Matrix (page 10)

    • Gel-like consistency with high concentration of soluble proteins (e.g., Krebs cycle enzymes)
    • Contains mitochondrial DNA (~1% of total cellular DNA content).
    • Contains free ribosomes which are smaller than cytoplasmic ribosomes.

    Mitochondrion: mtDNA (page 11)

    • mtDNA encodes 13 mitochondrial polypeptides, 2 rRNA, and 22 tRNA.
    • mtDNA has a high mutation rate, leading to heteroplasmy.
    • Lower fidelity of mitochondrial DNA replication, and inefficent DNA repair.
    • Useful in estimating dates of recent evolutionary events.

    Mitochondrion: Inheritance (page 12, 13)

    • Mitochondrial inheritance is uniparental (maternal) in both animals and plants.
    • Typical human oocytes contain ~100,000 copies of mtDNA.
    • Sperm mtDNA is degraded during maturation.
    • Sperm mitochondria are eliminated from the fertilized egg cell by autophagy.
    • Germ-line mitochondrial mutations are passed to the next generation by the mother.

    Mitochondrion: Biparental Inheritance (page 14)

    • While maternal inheritance of mtDNA is the norm, very rare instances of biparental mtDNA transmission have been observed in specific families.
    • These cases could potentially disrupt the idea of strict maternal mitochondrial heritage.

    Mitochondrion: Mitochondrial Protein Import (pages 15-17)

    • Mitochondrial proteins are synthesized on cytosolic ribosomes and imported by targeting signals.
    • Key translocator complexes: TOM (outer membrane), SAM (outer membrane), TIM (inner membrane) mediate the process.
    • OXA complex facilitates membrane protein insertion.
    • Chaperone interaction (e.g., hsp70 family) helps in precursor protein processing and insertion.

    Mitochondrion: Dynamic Organelles (page 18)

    • Mitochondria can fuse (or merge) and/or split.
    • Typically associated with the microtubular cytoskeleton.
    • Endoplasmic reticulum participates in mitochondrial division, starting the process

    Mitochondrion: Video - Mitochondrial Dynamics (page 19)

    • Video demonstrates mitochondria labeled with fluorescent proteins fusing and/or dividing.

    Mitochondrion: Mitochondrial Degradation (pages 20-21)

    • Nonfunctional mitochondria undergo organized degradation (mitophagy).

    • Inefficient protein import is linked to the loss of electrochemical gradient, and accumulation of PINK1 (a protein kinase) on the outer membrane.

    • PINK1 phosphorylates ubiquitin and regulator Parkin which initiates autophagosome formation.

    • Mutations in PINK1 and Parkin are linked to Parkinson's disease related to dysfunctional mitochondria accumulation.

    Mitochondrion (page 22):

    • Role in respiration and the urea cycle.
    • Involvement in lipid biosynthesis and apoptosis.
    • Regulates cytosolic Ca2+ concentration

    Aerobic Metabolism

    • Glycolysis: Glucose metabolism to pyruvate is initiated outside the mitochondria, and converts glucose into chemical energy.

    • Pyruvate and/or Fermentation: Oxygen availability is key; with oxygen (aerobic), pyruvate enters mitochondria; without oxygen (anaerobic), fermentation begins.

      • Decarboxylation: Pyruvate is converted into Acetyl CoA with the release of CO2 and creation of NADH from NAD+.
    • TCA Cycle (Citric Acid Cycle): Acetyl CoA proceeds through the TCA cycle in the mitochondrion, producing NADH and FADH2.

    • **Electron Transport Chain (ETC): ** High-energy electrons from NADH and FADH2 are transferred along the ETC, generating a proton gradient.

    Electron Transport Chain (pages 28-31)

    • 4 complexes and ubiquinone and cytochrome c support electron transfer.
    • Ubiquinone acts as an electron carrier.
    • Cytochrome c is a peripheral protein.
    • The ETC passes electrons to molecular oxygen forming water, releasing energy that is utilized for ATP synthesis.
    • The process conserves free energy through proton translocation across the inner membrane.

    Aerobic Metabolism: ATP Synthesis (pages 32-33)

    • The electrochemical proton gradient drives ATP synthase.
    • ATP synthase is a protein complex comprising F1 (spherical head) and F0 (basal portion), embedded in the membrane.
    • Proton flow through ATP synthase facilitates ADP to ATP phosphorylation.
    • Approx. 36 ATP molecules are generated from one glucose molecule.

    Mitochondrial Apoptosis (page 34)

    • Caspases are proteases, activated only during apoptosis.
    • Two main activation pathways, extrinsic and intrinsic.

    Mitochondrial Diseases (page 35)

    • Heterogeneous group of disorders caused by mtDNA or nuclear gene mutations.
    • Often associated with neuromuscular symptoms (Mitochondrial Myopathies).
    • Maternal inheritance often but not always the case.

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