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Questions and Answers
What is the primary reason for the destruction of paternal mitochondria after fertilization?
What is the primary reason for the destruction of paternal mitochondria after fertilization?
What is the characteristic of human mitochondrial diseases?
What is the characteristic of human mitochondrial diseases?
What is the consequence of mitochondrial mutations in somatic cells?
What is the consequence of mitochondrial mutations in somatic cells?
What is the pattern of transmission of human mitochondrial diseases?
What is the pattern of transmission of human mitochondrial diseases?
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What is the significance of heteroplasmy in mitochondrial disease?
What is the significance of heteroplasmy in mitochondrial disease?
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What is the mitochondrial gene mutated in Leber hereditary optic neuropathy?
What is the mitochondrial gene mutated in Leber hereditary optic neuropathy?
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What is the consequence of a high ratio of mutant to normal mitochondria in a cell?
What is the consequence of a high ratio of mutant to normal mitochondria in a cell?
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What is the goal of the three-parent baby technology?
What is the goal of the three-parent baby technology?
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What is the effect of high oxygen consumption on mitochondrial DNA?
What is the effect of high oxygen consumption on mitochondrial DNA?
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What is the significance of ubiquitin in the destruction of paternal mitochondria?
What is the significance of ubiquitin in the destruction of paternal mitochondria?
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What is the primary function of mitochondria?
What is the primary function of mitochondria?
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How many base pairs are present in the human mitochondrial DNA?
How many base pairs are present in the human mitochondrial DNA?
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What is the primary source of mitochondrial proteins?
What is the primary source of mitochondrial proteins?
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What is the range of variation in the size of mitochondrial genomes?
What is the range of variation in the size of mitochondrial genomes?
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What is the primary function of the 13 polypeptides encoded by mitochondrial DNA?
What is the primary function of the 13 polypeptides encoded by mitochondrial DNA?
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What is the size of mitochondrial genomes in plants?
What is the size of mitochondrial genomes in plants?
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What is the main reason for maternal inheritance of mitochondria in animals?
What is the main reason for maternal inheritance of mitochondria in animals?
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Which of the following organisms exhibits paternal inheritance of mitochondria and chloroplasts?
Which of the following organisms exhibits paternal inheritance of mitochondria and chloroplasts?
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What is the primary mechanism of maternal inheritance in Drosophila?
What is the primary mechanism of maternal inheritance in Drosophila?
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In which type of plants is biparental inheritance of mitochondria and chloroplasts observed?
In which type of plants is biparental inheritance of mitochondria and chloroplasts observed?
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What is the primary reason for maternal inheritance of mitochondria in mammals?
What is the primary reason for maternal inheritance of mitochondria in mammals?
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Which of the following is NOT a mechanism of maternal inheritance of mitochondria in animals?
Which of the following is NOT a mechanism of maternal inheritance of mitochondria in animals?
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Which species exhibits lack of entry of sperm mitochondria as a mechanism of maternal inheritance?
Which species exhibits lack of entry of sperm mitochondria as a mechanism of maternal inheritance?
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What is responsible for destroying mitochondrial DNA in sperm during maturation in Drosophila?
What is responsible for destroying mitochondrial DNA in sperm during maturation in Drosophila?
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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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Description
Learn about the destruction of sperm mitochondria after fertilization and the maternal inheritance of mitochondria in humans, including the transmission of mitochondrial diseases.