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Updated: Feb 25 2023


  • Overview
    • Overview
      • found in high concentrations in metabolically active cells
    • Cell biology
      • double membrane structure
        • outer membrane
          • high permeability due to high concentration of porins
        • inner membrane
          • impermeable to ions and molecules
          • folded into cristae to ↑ surface area
          • contains enzymes for electron transport chain (ETC)
      • inner matrix
        • contains circular DNA (mtDNA)
          • maternal inheritance as mitochondria in the zygote come from the egg
            • non-Mendelian inheritance
          • synthesized in continous polycistronic manner including
            • 13 coding genes
            • 2 ribosomal RNA modules
            • 22 transfer RNA segments
          • examples of mtDNA disorders
            • Leber's optic neuropathy
              • degeneration of retinal ganglion cells
              • progressive loss of central vision
              • eventual blindness
            • Pearson marrow-pancreas syndrome
          • some protein synthesis occurs here
          • most proteins are synthesized in cytoplasm and translocated to mitochondria
    • Function
      • oxidizes substrates
        • NADH/FADH2 → NAH+/FAD
      • generates ATP
        • converts NADH/FADH2 into ATP via ETC
      • stores Mg2+ and Ca2+
  • Mitochondrial Inheritance Pedigree
    • Affected males
      • transmit to none of their children
    • Affected females
      • transmit to all of their children
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