Cell Organelles: Mitochondria and Peroxisomes
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Questions and Answers

What is the primary component of the mitochondrial genome?

  • Linear DNA molecules
  • Circular DNA molecules (correct)
  • Chromosomal DNA
  • RNA molecules
  • How many proteins does the human mitochondrial genome encode that are involved in electron transport and oxidative phosphorylation?

  • 13 (correct)
  • 16
  • 8
  • 10
  • Which type of genes encode the proteins necessary for transcription and translation in mitochondria?

  • Plasmid genes
  • Nuclear genes (correct)
  • Mitochondrial genes
  • Cytoplasmic genes
  • What is the size of the human mitochondrial genome?

    <p>16 Kb</p> Signup and view all the answers

    Where are mitoribosomal proteins (MRPs) synthesized?

    <p>Cytosol</p> Signup and view all the answers

    What is the complete mitoribosome complex called?

    <p>55S monosome</p> Signup and view all the answers

    Which mitochondrial structure interacts with the translocase OXA1L?

    <p>Inner mitochondrial membrane</p> Signup and view all the answers

    What is primarily encoded by the mitochondrial genome apart from proteins?

    <p>tRNA and rRNA</p> Signup and view all the answers

    What is the main goal of mitochondrial replacement techniques?

    <p>To avoid transmission of mitochondrial diseases to offspring</p> Signup and view all the answers

    How many proteins are estimated to be encoded by the nuclear genome for mammalian mitochondria?

    <p>1,500</p> Signup and view all the answers

    What is the primary function of the Tom complex?

    <p>To direct protein translocation across the outer membrane</p> Signup and view all the answers

    Which sequence is crucial for directing proteins to the mitochondrial matrix?

    <p>Amino-terminal presequence</p> Signup and view all the answers

    What process converts glucose into pyruvate in animal cells?

    <p>Glycolysis</p> Signup and view all the answers

    What role does the electrochemical potential play in mitochondrial protein import?

    <p>It aids in the translocation of matrix proteins across the inner membrane.</p> Signup and view all the answers

    What is the primary role of the enzymes within the citric acid cycle?

    <p>To oxidatively degrade carbohydrates and fatty acids</p> Signup and view all the answers

    Which process is NOT associated with mitochondrial replacement techniques?

    <p>Cloning of mitochondrial DNA</p> Signup and view all the answers

    Which protein complex is responsible for transferring proteins to the inner membrane of mitochondria?

    <p>Tim23</p> Signup and view all the answers

    Which of the following statements about the inner mitochondrial membrane is true?

    <p>It contains 70% proteins involved in oxidative phosphorylation.</p> Signup and view all the answers

    How is the energy used for ATP synthesis generated during oxidative phosphorylation?

    <p>Through the proton gradient across the inner membrane</p> Signup and view all the answers

    Which of the following describes the structure of mammalian mitochondria?

    <p>A double membrane system</p> Signup and view all the answers

    What is the fate of pyruvate after glycolysis?

    <p>It is oxidized to acetyl CoA in the mitochondria.</p> Signup and view all the answers

    Which type of molecules can freely pass through the outer mitochondrial membrane?

    <p>Small molecules and ions</p> Signup and view all the answers

    What type of relationship is believed to have led to the evolution of mitochondria?

    <p>Symbiotic relationship</p> Signup and view all the answers

    Which statement correctly describes the fate of the high-energy electrons from NADH and FADH2?

    <p>They are transferred to oxygen through transporters.</p> Signup and view all the answers

    What is the primary function of mitochondria in cells?

    <p>Generate ATP from the breakdown of carbohydrates and fatty acids</p> Signup and view all the answers

    What unique feature do mitochondria possess that distinguishes them from peroxisomes?

    <p>Capable of protein synthesis from their own DNA</p> Signup and view all the answers

    How are most mitochondrial proteins synthesized?

    <p>On free ribosomes in the cytoplasm</p> Signup and view all the answers

    What structural feature increases the surface area of the inner mitochondrial membrane?

    <p>Cristae</p> Signup and view all the answers

    What is the role of enzymes contained within peroxisomes?

    <p>Facilitate metabolic pathways such as fatty acid breakdown</p> Signup and view all the answers

    Which compartments of mitochondria are primarily involved in oxidative metabolism?

    <p>Mitochondrial matrix and inner membrane</p> Signup and view all the answers

    What process describes the dynamic changes in mitochondria as they fuse and divide?

    <p>Fusion and fission</p> Signup and view all the answers

    What type of genetic material is found within the mitochondrial matrix?

    <p>Mitochondrial DNA coding for tRNA and rRNA</p> Signup and view all the answers

    What is the primary role of cardiolipin in the mitochondria?

    <p>It enhances the efficiency of oxidative phosphorylation.</p> Signup and view all the answers

    Which statement about peroxisomes is correct?

    <p>Human cells may contain between 100 and 1000 peroxisomes depending on activity.</p> Signup and view all the answers

    How are lipids transferred from the ER to the mitochondria?

    <p>At close contact points mediated by phospholipid transfer proteins.</p> Signup and view all the answers

    What distinguishes the enzymes found in peroxisomes from those in mitochondria?

    <p>Peroxisome enzymes resemble typical eukaryotic proteins.</p> Signup and view all the answers

    What is produced as a result of oxidative reactions in peroxisomes?

    <p>Hydrogen peroxide.</p> Signup and view all the answers

    Which of the following is NOT a characteristic of peroxisomes?

    <p>They generate energy through ATP synthesis.</p> Signup and view all the answers

    Cardiolipin is classified as which type of lipid?

    <p>Double phospholipid.</p> Signup and view all the answers

    Which structure in the cell is responsible for the synthesis of phospholipids that may end up in the mitochondrial membranes?

    <p>Endoplasmic reticulum (ER).</p> Signup and view all the answers

    What is the primary function of catalase in peroxisomes?

    <p>To break down hydrogen peroxide</p> Signup and view all the answers

    Which two signals are used for the targeting of internal peroxisomal proteins?

    <p>PTS1 and PTS2</p> Signup and view all the answers

    Which of the following is NOT involved in the import of matrix proteins into peroxisomes?

    <p>Pex19</p> Signup and view all the answers

    What is a key function of plasmalogens formed in peroxisomes?

    <p>Phospholipid synthesis for cell membranes</p> Signup and view all the answers

    How are most transmembrane proteins for peroxisomes synthesized?

    <p>Synthesized in the ER</p> Signup and view all the answers

    Which peroxin is specifically involved in recognizing the PTS1 signal?

    <p>Pex5</p> Signup and view all the answers

    What is one of the mechanisms through which new peroxisomes can form?

    <p>Budding from the ER</p> Signup and view all the answers

    Which of the following components are recognized by Pex19 during the import of peroxisomal membrane proteins?

    <p>Peroxisomal targeting signal (mPTS)</p> Signup and view all the answers

    Study Notes

    Mitochondria and Peroxisomes

    • Mitochondria are responsible for generating most of the cell's usable energy from the breakdown of lipids and carbohydrates.
    • Peroxisomes contain enzymes involved in various metabolic pathways, including fatty acid breakdown and photorespiration.
    • Proteins destined for mitochondria and peroxisomes are synthesized on free ribosomes in the cytosol, except for membrane proteins of the peroxisome.
    • Proteins import into target organelles, in the form of complete polypeptide chains.
    • Mitochondria contain their own genomes, which include genes transcribed and translated within the organelle itself.
    • Mitochondria are surrounded by a double membrane system, an inner and outer mitochondrial membrane separated by an intermembrane space
    • The inner membrane folds into numerous cristae, which extend into the mitochondrial matrix
    • The matrix and inner membrane are the primary functional compartments of mitochondria.
    • Mitochondria are dynamic and undergo fusion and fission.
    • The matrix contains mitochondrial genetic system and enzymes responsible for central reactions of oxidative metabolism.
    • The primary source of metabolic energy involves the breakdown of glucose and fatty acids.
    • The oxidation of acetyl CoA to CO2 is linked to the reduction of NAD+ and FAD to NADH and FADH2, respectively.
    • Most energy from oxidative metabolism is produced by oxidative phosphorylation in the inner mitochondrial membrane.
    • High-energy electrons from NADH and FADH2 are transferred to oxygen via membrane transporters.
    • The energy from electron transfer is converted into a proton gradient used to synthesize ATP.
    • The inner mitochondrial membrane has high protein content, is involved in oxidative phosphorylation, and maintains a critical proton gradient.
    • Outer membrane is completely permeable to small molecules via porins, which allow passage of molecules smaller than 1000 daltons.
    • The composition of the intermembrane space is similar to that of the cytosol.

    Genetic System of Mitochondria

    • Mitochondria evolved from bacteria.
    • Mitochondrial genome forms a circular DNA molecule and several copies per organelle.
    • Size of the mitochondrial genome varies between different species.
    • Mitochondrial DNA encodes essential components for oxidative phosphorylation (tRNA, rRNA, some proteins).
    • Other mitochondrial proteins are encoded by nuclear genes and transferred from ancestral mitochondrial genomes.
    • Human mitochondrial genome is only 16 kb, while yeast and plants have larger genomes
    • Human mitochondrial genome codes for 13 proteins, 16S and 12S ribosomal RNA, and 22 transfer RNAs.
    • Nuclear genes provide the rest of the proteins required for mitochondrial functions.

    Mitochondrial Diseases

    • Mitochondrial DNA mutations, primarily transmitted through the maternal line, are often harmful to the organelle.
    • Mitochondrial inheritance means defects in mtDNA are inherited from the mother.
    • Homoplasmy means the multiple copies of mitochondrial DNA in the cell are identical, while heteroplasmy means a combination of wild-type and mutated mitochondrial DNA.

    Mitochondrial Protein Internalization and Formation

    • Mammalian mitochondria contain about 1,500 proteins encoded by nuclear genome.
    • Nuclear-encoded proteins are synthesized by free ribosomes in the cytoplasm and transported into mitochondria.
    • Protein import is complex due to the double membrane of mitochondria.
    • Proteins with amino terminal pre-sequences are the most studied.
    • These pre-sequences bind to the Tom complex on the outer membrane for initial translocation.
    • Mitochondrial matrix proteins cross the inner membrane via the Tim23-complex, utilizing the electrochemical gradient.
    • Other proteins utilizing a transmembrane sequence laterally insert into the inner membrane via other transport mechanisms.
    • Matrix proteases remove pre-sequences.
    • Protein import through complex pathways in the matrix and inner membrane, necessitating chaperones

    Peroxisomes

    • Peroxisomes are small organelles (0.1-1 µm) with a membrane.
    • Human cells have a variable number (100-1000) depending on activity.
    • Peroxisomes lack a genome and utilize ribosomes in the cytoplasm for protein synthesis, while some proteins from the ER.
    • Peroxisome function includes various metabolic reactions and protein synthesis.
    • Peroxisomes are responsible for oxidation reactions that produce hydrogen peroxide.
    • Catalase, a key peroxisomal enzyme, breaks down hydrogen peroxide.
    • Peroxisomes oxidize branched and longer-chain fatty acids, along with other substrates.
    • Peroxisomes participate in plasmalogen synthesis (important membrane component of heart and brain).

    Peroxisome Assembly

    • Internal peroxisomal proteins synthesize in the cytosol, while the bulk of transmembrane proteins are synthesized in the ER.
    • Transmembrane proteins (peroxins, Pex proteins) are important for metabolite transport.
    • PTS1 (Ser-Lys-Leu) signal, is widely used for proteins targeting peroxisomes.
    • PTS2 (amino-terminal sequences of 9 aa) is another targeting designation.
    • Peroxins (Pex5, Pex7, etc), as cytosolic receptors, bind to PTS1/PTS2 to initiate import to the peroxisome.
    • The Pex complex anchors proteins to peroxisome membrane and helps them enter.

    Lipid Transfer and Lipids in Peroxisomes

    • Lipid transfer between ER and mitochondria happens at contact points and requires phospholipid transfer proteins to extract phospholipid molecules from ER and introduce them to the mitochondria.
    • Lipids can then transport via an aqueous environment.
    • Lipids move between the inner and outer mitochondrial membranes at contact points.
    • The majority of lipids in the mitochondrial membranes originate from the cytosol and undergo synthesis in the ER.
    • Phosphatidylethanolamine is produced from phosphatidylserine.
    • Cardiolipin, a four fatty acid chain phospholipid, is synthesized in the inner membrane and enhances oxidative phosphorylation.

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    This quiz covers the key functions and characteristics of mitochondria and peroxisomes within cells. You'll explore their roles in energy production, metabolism, and protein synthesis. Perfect for anyone studying cell biology or biochemistry!

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