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| 3111669411 | 5 major functions of mitochondrion | ATP production Regulate metabolism Cell death Calcium storage Biosynth of heme Cell signaling Urea cycle | 0 | |
| 3127336551 | What is a mitochondrial function? | ATP synth, heat, calcium storage, apoptosis | 1 | |
| 3111711007 | Major organs affected by mitochon disease | Eye Muscle GI Brain Heart | 2 | |
| 3111731310 | Mitochondrial proteome is different from organ to organ | Interpretation of mito events should include the proteome Mito mutations are dependent on the tissue. | 3 | |
| 3111736888 | Mito anatomy |  | 4 | |
| 3111740725 | Michondrial cross talk between nucleus | This communication is important for correct synthesis | 5 | |
| 3111744785 | Mitochondrial genome | 37 genes 13 proteins No introns dsDNA tRNA rRNA | 6 | |
| 3127395756 | What is the worst kinda mtDNA mutation? | tRNA or rRNA | 7 | |
| 3127385757 | Complexes coded by mtDNA | 1,3,4,5 (ATP synthase) |  | 8 |
| 3111787379 | MtDNA mutation rate | 15x higher than genomic There is cell threshold where more than 60% of bad mitos cause mutation effect | 9 | |
| 3127403596 | Copies of DNA per mito | Each mito has 2-10 copies of mtDNA | 10 | |
| 3127406319 | When do mitos do replication | They do fission in mitosis or meiosis | 11 | |
| 3111807057 | MtDNA homoplasmy | Same mutation/non mutation mitos in cell | 12 | |
| 3111851112 | IVF | Take out mothers nucleus and put it in non mutated donor cell. | 13 | |
| 3111811742 | MtDNA heteroplasmy | Mix of mutated and non-mutated MtDNA in cell. These non-mutated mitos can keep you alive. | 14 | |
| 3111863650 | What is the role of the nuclear genome in mitochondria | Cooperates with mtDNA to make multi subunit complexes of ETC Protein transport machinery Encodes TFs in mito |  | 15 |
| 3121447607 | Matrix enzymes of mito | These are all nuclear mRNA made Ox phosph enzymes are mix of nuclear and mito DNA | 16 | |
| 3111892547 | How do get nuclear proteins into matrix? | 90% of mito proteins have to be imported Targeting or presequence for this to occur. 20-70 aas. |  | 17 |
| 3111966508 | Hsp70 | Unfolding factor in mito. Needs ATP | 18 | |
| 3111953678 | TOMs vs TIMs | Import receptors on OMM | 19 | |
| 3111957291 | SAM | Sorting of assembly machinery | 20 | |
| 3112021302 | Possible sources of mtDNA mutations | Nuclear DNA - mendelian Cytoplasmic mtDNA - maternal X linked |  | 21 |
| 3112074019 | Why are there mt mutations? | MtDNA attaches to IMM. ROS. MtDNA lacks protective histones MtDNA has limited repair systems | 22 | |
| 3112091118 | Missense mutation | Aa substitution Leber's hereditary optic neuronpathy | 23 | |
| 3127486514 | Biogenesis mutations | tRNA point mutations Affects protein synth | 24 | |
| 3112095460 | tRNA mutations from mtDNA | MERRF MELAS More deleterious than the 13 polypeptides in comlpex | 25 | |
| 3112102201 | Insertion deletion mutations of mtDNA | No family history KSS PEO | 26 | |
| 3127490922 | Kearns Sayre syndrom | Indel mt DNA mutation | 27 | |
| 3127491946 | Progressive external opthalmoplegia | Indel mt DNA mutation | 28 | |
| 3112107845 | Fusion and fission in mtDNA | This is for quality control of mt proteins. Remove damaged mitos by combining them with good ones |  | 29 |
| 3112111424 | PGC-1 alpha | Key regulator of mt protein synth Co transcriptional activator. This is nuclear gene. Turned on my AMP kinase Then it turns on mt genes Turns on when you are cold via PKA |  | 30 |
| 3127533452 | PGC-1 alpha neg feedback | Energy charge will turn off itself |  | 31 |
| 3112123146 | AMP kinase | Helps to integrate energy demands with cellular metabolism Stops ATP consumption Starts ATP generation Memorize this slide |  | 32 |
| 3127547189 | What does AMP do? | Stimulates glycogenolysis and glycolysis Also activates AMP kinase when makes more ATP | 33 | |
| 3121473526 | What activates PGC-1 alpha? | AMP kinase which was activated by AMP | 34 | |
| 3112137813 | AMP kinase inhibtion | ATP | 35 | |
| 3112144169 | What activates mt biogenesis? | Cell is making ATP in low E state AMPK is activated. Activates PGC-1alpha |  | 36 |
| 3127569683 | Type 2 diabetes drug (Mephormin) | Turns on AMPK More mtDNA genes | 37 | |
| 3112152067 | Mitophagy | This removes damaged mitos Bad mitophagy means you have brain disease Bad fusion and fission means you have brain issue too | 38 | |
| 3112153818 | Apoptosis and mt | This is important for fighting viruses Cyt C release from Bax and Bak | 39 | |
| 3127585640 | What is committed step of apoptosis? | Cyt C release is committed step of apoptosis |  | 40 |
| 3112161572 | Cyt C | Release is controlled by bcl-2 (Bax or Bak) or (Bcls) Thought to have been a humble protein only involved in ETC | 41 | |
| 3112165514 | Pro apoptotic Bcl-2 | Bax and Bak | 42 | |
| 3112189198 | Pro survival Bcl-2 | Bcl-2, Bcl-W, Bcl-xl, Mcl1, A1 | 43 | |
| 3127611589 | Disease of aging | Mitos are involved. Reactive oxidative species increase with time. This causes problems. mtDNA mutations build up | 44 |
