Updated: 9/6/2020

Mitochondria

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Mitochondria Structures 
 
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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