Non-Medellin Inheritance Part 2 (Hard)
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Questions and Answers

What is the primary reason for the destruction of paternal mitochondria after fertilization?

  • Paternal mitochondria are larger than maternal mitochondria
  • Paternal mitochondria have a different DNA structure
  • Paternal mitochondria are unable to replicate
  • Paternal mitochondria are modified by ubiquitin, a small regulatory protein (correct)
  • What is the characteristic of human mitochondrial diseases?

  • They are caused by environmental factors
  • They are inherited from the father
  • They are acute and affect all cells
  • They are chronic and affect cells requiring high levels of ATP (correct)
  • What is the consequence of mitochondrial mutations in somatic cells?

  • They are eliminated from the cell
  • They are immediately repaired by mitochondrial DNA repair mechanisms
  • They accumulate as a person ages (correct)
  • They have no effect on the cell
  • What is the pattern of transmission of human mitochondrial diseases?

    <p>Strict maternal inheritance</p> Signup and view all the answers

    What is the significance of heteroplasmy in mitochondrial disease?

    <p>It can lead to varying symptoms within a given family</p> Signup and view all the answers

    What is the mitochondrial gene mutated in Leber hereditary optic neuropathy?

    <p>Any of the above</p> Signup and view all the answers

    What is the consequence of a high ratio of mutant to normal mitochondria in a cell?

    <p>The cell may exceed a threshold value and express disease</p> Signup and view all the answers

    What is the goal of the three-parent baby technology?

    <p>To reduce the risk of mitochondrial disease transmission</p> Signup and view all the answers

    What is the effect of high oxygen consumption on mitochondrial DNA?

    <p>It leads to DNA damage and free radical formation</p> Signup and view all the answers

    What is the significance of ubiquitin in the destruction of paternal mitochondria?

    <p>It targets paternal mitochondria for destruction</p> Signup and view all the answers

    What is the primary function of mitochondria?

    <p>Oxidative phosphorylation</p> Signup and view all the answers

    How many base pairs are present in the human mitochondrial DNA?

    <p>17,000 bp</p> Signup and view all the answers

    What is the primary source of mitochondrial proteins?

    <p>Nuclear DNA</p> Signup and view all the answers

    What is the range of variation in the size of mitochondrial genomes?

    <p>400-fold</p> Signup and view all the answers

    What is the primary function of the 13 polypeptides encoded by mitochondrial DNA?

    <p>Oxidative phosphorylation</p> Signup and view all the answers

    What is the size of mitochondrial genomes in plants?

    <p>Fairly large</p> Signup and view all the answers

    What is the main reason for maternal inheritance of mitochondria in animals?

    <p>Destruction of Sperm Mitochondrial DNA Prior to Fertilization</p> Signup and view all the answers

    Which of the following organisms exhibits paternal inheritance of mitochondria and chloroplasts?

    <p>Gymnosperms (Plants)</p> Signup and view all the answers

    What is the primary mechanism of maternal inheritance in Drosophila?

    <p>Destruction of Sperm Mitochondrial DNA Prior to Fertilization</p> Signup and view all the answers

    In which type of plants is biparental inheritance of mitochondria and chloroplasts observed?

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

    What is the primary reason for maternal inheritance of mitochondria in mammals?

    <p>Lack of Entry of Sperm Mitochondria</p> Signup and view all the answers

    Which of the following is NOT a mechanism of maternal inheritance of mitochondria in animals?

    <p>Biparental Inheritance</p> Signup and view all the answers

    Which species exhibits lack of entry of sperm mitochondria as a mechanism of maternal inheritance?

    <p>Chinese hamsters (Cricetulus griseus)</p> Signup and view all the answers

    What is responsible for destroying mitochondrial DNA in sperm during maturation in Drosophila?

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

    Study Notes

    Destruction of Sperm Mitochondria After Fertilization

    • Paternal mitochondria that enter the egg are modified by ubiquitin, a small regulatory protein that targets organelle destruction
    • This process occurs in most mammals, including humans

    Maternal Inheritance of Mitochondria

    • Human mtDNA is transmitted from the female parent to offspring via the cytoplasm of the egg
    • Therefore, the transmission of human mitochondrial diseases follows a strict maternal inheritance pattern

    Human Mitochondrial Diseases

    • Over 200 human mitochondrial diseases have been identified
    • These diseases are typically chronic degenerative disorders affecting cells requiring high levels of ATP, such as nerve and muscle cells
    • Examples of human mitochondrial diseases include:
      • Leber hereditary optic neuropathy, caused by mutations in mitochondrial genes encoding respiratory chain proteins
      • Neurogenic muscle weakness, caused by mutations in the ATPase6 gene
      • Mitochondrial myopathy and maternal myopathy and cardiomyopathy, caused by mutations in genes encoding tRNA for leucine

    Heteroplasmy in Mitochondrial Disease

    • Heteroplasmy is an important factor in mitochondrial disease, where cells can contain a mixed population of mitochondria, some with disease-causing mutations and others without
    • The ratio of mutant to normal mitochondria can exceed a threshold value, leading to disease symptoms that may vary widely within a given family

    Three Parent Babies

    • A new reproductive technology has emerged to prevent the transmission of mitochondrial diseases from mothers to their offspring
    • This technology was pioneered by John Zhang and colleagues in 2016, and involves replacing the mother's mitochondria with those from a donor woman

    Mechanisms of Maternal Inheritance

    • There are three mechanisms responsible for maternal inheritance of mitochondria:
      • Lack of entry of sperm mitochondria into the egg
      • Destruction of sperm mitochondrial DNA prior to fertilization
      • Destruction of sperm mitochondria after fertilization

    Sizes of Organellar Genomes

    • The sizes of organellar genomes vary greatly among different species, with a 400-fold variation in the size of mitochondrial genomes
    • Mitochondrial genomes are fairly small in animals, intermediate in size in fungi and protists, and fairly large in plants

    Mitochondrial DNA

    • The main function of mitochondria is oxidative phosphorylation, which generates ATP (adenosine triphosphate) for cellular reactions
    • Human mitochondrial DNA (mtDNA) consists of only 17,000 bp and includes genes for rRNA, tRNA, and 13 polypeptides involved in oxidative phosphorylation
    • Most mitochondrial proteins are encoded by genes in the nucleus and are made in the cytoplasm before being transported into the mitochondria

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    Learn about the destruction of sperm mitochondria after fertilization and the maternal inheritance of mitochondria in humans, including the transmission of mitochondrial diseases.

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