Mitochondria and Peroxisomes

Mitochondria and Peroxisomes

1. Mitochondria

   • associations
     • microtubules
   • configurations
     • orthodox
       • prominent cristae
       • prominent matrix
       • small IMS volume
       • low ATP synthesis
     • condensed
       • prominent IMS
       • small matrix
       • max ATP synthesis
   • distribution
     • high energy demand
       • higher mitochondria
   • structural features
     • MOM
       • outer membrane
         • porin
           • aqueous channels
             • small diffusion
         • TOM
           • receptor
             • transports through MOM
               • unfloded
                 • Hsp70, MSF
           • TIM
             • binds TOM
               • targeted to matrix, MIM, IMS
                 • gradient dependent
           • + alpha-helix
             • N-ter,minus
               • binds cis-site
                 • goes through pore
                   • binds trans-site
                     • inner surface
     • IMS
       • cytochrome C
       • B2
     • MIM
       • ETC
         • ATP synthesis
           • ATP Synthase
             • F1F0
             • F0
               • integral membrane complex
                 • proton channel
             • F1
               • peripheral complex
                 • actual ATP synthase
                   • spins -- mechanical energy
                     • transferred to chemical energy
         • complexes
           • transport NADH, FADH
             • generated in Krebs cycle
           • creates proton gradient
             • drives ATP synthesis
       • cristae
         • increase SA
     • matrix
       • Krebs cycle
         • enzhymes
       • mito DNA
       • ribosomes
       • electron dense granules
         • Ca/phospholipids
           • Ca sequestration
             • low Ca in cytoplasm
             • Ca entry
               • coupled to H+ entry
                 • symport
   • genome
     • 2 rRNAs
     • 22 tRNAs
       • unique codons
         • unique tRNA specificity
     • 13 proteins
     • ETC complexes
     • ATP synthase
     • inheritance
       • maternal
   • divison
     • fission
   • morphologies
     • tubulovesicular
       • tubular cristae
       • steroid secreting cells
     • lamellar
       • most cells
       • lamellar cristae
   • disease
     • mutations
       • nuuclear DNA
       • mito DNA
       • missense
         • Leber's Hereditary Optic Neuropathy
       • biogenesis
         • MERRF
         • MERLAS
         • tRNA muttions
           • affect protein synthesis

2. Peroxisomes

   • function
     • oxygen utilization
       • breakdown FAs
       • alcohol
       • oxidases
         • generate H2O2
     • detox
       • catalase
         • liver, kidney
         • removes H2O2
   • biogenesis
     • response
       • environmental stimuli
         • growth and survival
     • PPARs
       • translocate to nucleus
         • bind promoters
           • steeroids
           • thyroid homrone
           • retinoic acid
           • peroxisomal enzymes
             • increased synthesis
     • import proteins
       • increase proteins
         • budding
           • new peroxisome
           • off ER
         • fission
       • target sequences
         • PTS1
           • C-terminal
         • PTS2
           • N-terminal
   • disease
     • impaired transport
       • Zellweger's
         • Pex2
           • protein import
             • peroxisome proteins remain in cytoplasm
         • severe defects
           • liver, kidney
             • death
       • X-linked Adenoleukodystrophy
         • ADL gene
           • imports long-chain fatty acyl CoA synthase
             • oxidation of FA
               • FA build up
         • cellular damage
           • neurons