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Mitochondria, Peroxisomes (microbodies) - Coggle Diagram

- - - - Cardiolipid: four fatty acid chain that allows for conc. gradient due to its high impermeability. Makes majority of inner membrane
      - Energy used for many processes
        
        ATP production
        
        Pyruvate transport into matrix
        
        Inorganic phosphate into matrix
        
        Used for ADP/ATP translocase activity.
    - - Complex 1
        
        Takes e- off of NADH
        
        Toxin: Rotenone
        
        Inhibits complex 1
        
        Inhibition of this complex only decreases efficiency of ETC
        
        Complex 2 can still get e-
        
        Both pump protons into intermembrane space
      - Complex 2
        
        Takes off e- off of FADH
        
        Ubiquinone- e- carrier of both complex 1 and 2
      - Complex 3
        
        Takes e- from ubiquinone and continues pumping
        
        Toxin: Anti-mycin: inhibits complex 3
        
        This blocks oxygen consumption bc it stops the ETC.
        
        Cytochrome C carries e- to Complex 4
      - Complex 4
        
        Dumps e- into oxygen. Water byproduct is made here.
        
        Oxygen must be taken in to dump the used up e-.
        
        Toxins: CO, Azide, and Cyanide
        
        Inhibits this complex
        
        Blocks oxygen consumption and stops ETC
      - Complex 5: ATP synthase
        
        Uses chemical and voltage gradient to move H+ back to matrix and use the energy from this to make ATP.
        
        Toxin: Oligomycin
        
        Loss of ATP production
        
        Any loss of ATP production will increase fermentation
        
        Increased fermentation results in lactic acid buildup. Lactic acidosis
        
        ADP/ATP translocase moves the ATP and stuff in/out the matrix.
        
        Inhibition prevents ATP synthesis
        
        Toxins: ABC
        
        Bongkrek acid
        
        Atractyloside
        
        carboxyatractyoloside
    - - Sites for escape: Complex 1 + 3; Ubiquinone
      - Manganese superoxide dismutase (Mn-SOD) in matrix converts the free radical to H2O2
        
        Catalase or glutathione peroxidase can degrade it into water and oxygen.
        
        H2O2 can also react again with free radical.
  - - - Decreases ATP efficiency but heat is produced.
        
        Chemicals
        
        Thermogenin (UPC1)
        
        Found in brown fat
        
        2,4 dinitrophenol (DNP)
        
        Synthetic chemical; lethal at high doses.
      - Increased Oxygen consumption
- - - - Machinery for mitochondrial gene expression are here
        
        Mitochondrial ribosomes and tRNA.
      - No histones and introns = susceptible to damage
        
        Mutation often impair oxidative phosphorylation enzymes.
        
        Ex of disease: Myoclonic epilepsy
      - Relaxed codon usage: no wobble used
      - Mutation inheritance: Homoplasmy or Heteroplasmy
        
        Homoplasmy: all mitochondrial genome are identical; still difficult to predict disease bc penetrance is variable
        
        Disease: Hereditary optic neuropathy
        
        Heteroplasmy: Presence of normal and mutated mtDNA; results in variable mitochondrial disease
        
        Heteroplasmy threshold: Mutation is functionally recessive and phenotype won't show if there is sufficient expression from the normal mtDNA.
        
        Ex. Genetic bottleneck: During selection of oocytes, a select number of mtDNA are selected. Could mean variance in disease phenotype as a result.
- - - - Plasmalogen required for myelin sheathes.
- - - - S/Sx: Craniofacial abnormalities, neurological and liver dysfunction. Elevated FA in blood. Most severely affected die in the first year.
  - - - S/Sx: Vision and olfaction loss; bone abnormalities in hands/feet; progressive muscle weakness and atrophy
      - Phytanic acid comes from diet and must undergo alpha oxidation prior to beta oxidation. Build up neg affects myelin sheath growth and fx.