mitochondria Flashcards
mitochondria
- Double Membrane
o Inner Membrane folded many
times to incr. SA
Generate most of the ATP in cells
Inner most space is called the Matrix
Contain their own DNA
Generally inherited maternally
Mitochondrial genes
The human mitochondrion contains 5–10 identical, circular molecules of DNA.
mutations in mtDNA cause human diseases
- Mutations in 12 of the 13 polypeptide-encoding mitochondrial genes have been found to cause human disease.
- disorders of the muscles and brain are the most common. Perhaps this reflects the great demand for energy of both these organs. (Although representing only ~2% of our body weight, the brain consumes ~20% of the energy produced when we are at rest.)
- Some of these disorders are inherited in the germ-line. The mutant gene is received from the mother because none of the mitochondria in sperm survives in the fertilized egg.
- Other disorders are somatic; that is, the mutation occurs in the somatic tissues of the individual.
Example of mutation
- exercise intolerance
- A number of humans who suffer from easily-fatigued muscles turn out to have mutations in the cytochrome b gene.
- Only the mitochondria in their muscles have the mutation; the mtDNA of their other tissues is normal. Presumably, very early in their embryonic development, a mutation occurred in a cytochrome b gene in the mitochondrion of a cell destined to produce their muscles.
What is the mixing of mutant and normal DNA Called
heteroplasmy.
What is is it Called with the mitochondria carries all mutant genomes
homoplasmy (a phenomenon resembling genetic drift).
Why do mitochondria have their own genome?
Simply put, to allow redox regulation of gene expression**
- Many of the features of the mitochondrial genetic system resemble those found in bacteria, strengthening the theory that mitochondria are the evolutionary descendants of a bacterium))
- However, many of the genes needed for mitochondrial function have since moved to the nuclear genome.
-The recent sequencing of the complete genome of Rickettsia prowazekii has revealed a number of genes closely related to those found in mitochondria through oxidative level phosphorylation. As they produce ATP in the same way and lack genes to produce it through glycolysis