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16 Cards in this Set
- Front
- Back
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What's the chief characteristic of mitochodrial DNA?
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It's a double stranded, circular DNA molecule.
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How many mitochondrial polypeptides are involved with OXPHOS?
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13
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How many polypeptides are inolved with OXPHOS?
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About 90! (Don't forget extra-mitochondrial polypeptides)
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How many genes are in the mitochondrial genome?
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37 (rRNAs, tRNAs, and OXPHOS polypeptides)
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Is mitochondrial division coordinated with cell division?
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No. Mitochondrial division is independent of cell division.
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What term is used to describe the believed origin of mitochondria?
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endosymbiosis
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What is the inheritance pattern of mitochondrial DNA?
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It's exculsively maternal in origin.
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What's the difference between mitochondrial inheritance vs. mitochondrial disorders?
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Mitochondrial inheritance relates to the 37 genes of mtDNA, while mitochondrial disorders can be related to nDNA
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How is a pedigree for a mitochondrial inheritance disease different than an X-linked dominant disease?
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Affected mother's always transmit the disorder, but affected father's never do.
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Do mitochondrial inheritance patterns always follow a classical pattern?
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No, there can be variable expressivity dependent on how egg cells develop.
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What is an example of a mitochondrial disorder that is due to a missense mutation in a protein-encoding mtDNA gene?
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Leber hereditary optic neuropathy (LHON)
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What mitochondrial disorder is due to a single-base mutation in a tRNA gene?
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MERFF (myoclonis epilepsy with ragged red fibers)
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What are some other mechanisms of mitochondrially inheritted disorders?
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duplications of deletions of mtDNA, point mutations, deletions
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What important mitochondrial disorder of OXPHOS is nuclear in origin?
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Leigh disease
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How many polypeptides are involved with the OXPHOS system?
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100, 13 of which come from mtDNA
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How are OXPHOS disorders in nDNA typically inherited?
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autosomal recessive
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