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11 Cards in this Set

  • Front
  • Back
Does nDNA or mtDNA play a larger role in mitochondrial proteins?
-nDNA
Of the 5 complexes that are involved OXPHOS, which has no mtDNA?
- complex 2
What are the 5 unique features of mitochondrial DNA/protein synthesis?
- maternally inherited
heteroplasmic
- phenotype depends on number of mutants/cell and type of cell
- mtDNA acquires mutations faster
- a large deletion can knock out mtRNA and have my affects on gene products
explain replicative segregation
- in heterplasmic mitochondria (mix of mutant and normal mtDNA), mtDNA can shift during cell replication, causing varying amounts of normal vs mutant mtDNA to end up in the daughter cells
What are the 5 systems that are most dependent on mitochondria from most to least dependent?
- CNS
- skeletal muscle
- heart
- kidney
- liver
What are the 6 points of support for the symbiotic theory?
- double membrane
- bacterial lipids similar to mitochondrial
- mt ribosome RNA similar to bacterial
- circular DNA
- similar G/C ratio
- mito division like bacterial
Where does electron transport and oxidative phosphorylation take place?
- Inner membrane of mitochondria
How can environmental insults affect mitochondrial disease?
- may explain why different offspring manifest different degrees of the same disorder
- chronic poisoning of OXPHOS, hypoxia, muscle ischemia
What do cytochrome oxidase negative cells have to do with mitochondrial diseases?
- cytochrome oxidase negative cells increase in number as we get older and mutations accumulate leading to loss of functional cells
What signs are indicative of mitochondrial diseases?
- atypical presentation with a common disorder
What are the 3 important parts of OXPHOS complexes?
1) generates energy from ADP by chemiosmotic coupling
2) ATP makes PCr which can be used for muscular contractions
3) ATP moves ions, drives chemical reactions, and is involved in cellular repair