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10 Cards in this Set
- Front
- Back
Respiratory Quotient
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1.0 for glucose
.70 for palmitic acid Protein is somewhere in between. |
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Energy Storage
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Chemical Energy: ATP-->ADP>AMP; GTP (protein synthesis); UTP- nucleotide activation of small molecules; Creatine phosphate- energy storage in muscles. Made by CPK.
Electrical: NAD+/NADH- main carrier for electrical energy produced by reduction-oxidation reactions that are part of catabolism. NADH and NADPH- anabolic reactions. FAD/FADH2, less energy than NADH or NADPH. TCA cycle created. |
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Mitochondrial Structure
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Outer membrane: some enzymatic activities. Relatively impermeable.
Innter membrne: Shuttles for reduced electron carriers, ATP, pyruvate, citrate, area for ox phos. 70% membrane. Matrix: PDH complex, TCA cycle, fatty acid oxidation, urea, etc |
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Mitochondrial energy production:
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3 Stages:
1. Generation of an activated acetyl-CoA from AA, Carb, fat. 2. TCA cycle, which generates electron carriers NADH and FADH2. 3. Oxidative phosphorylation. |
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Electron Chain
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Purpose: Converstion of electrical to chemical energy.
Location: IMM NADH and FADH2 converted to energy via ATP. Regulation: NADH, FADH2, and ADP: uncoupling proteins |
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Organization of the ETC
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5 complexes: I, II, III, IV, and V. V is ATP synthase.
Complex I: NADH dehydrogenase: FMN-FMNH2, NADH is oxidized. Hydrogen atoms are transfered to CoQ10 (II), ubiquinone. Moves from I--III-IV. These are cytochromes with heme groups. At IV, O2 accepts Hydrogen. Reduced to H20. This is used to make ATP via ATP Synthase. 3 ATP per NADH, because 3 H have been pumped. FADH2 only 2. |
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Regulation of ETC:
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Regulated by availability of NADH and FADH. ADP> Thermogenin and other uncoupling proteins. Toxins: aspirin, DNP, inhibitors.
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ENERGY Balance Sheet
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Aerobic- Glycolysis:
Produced: 2ATP, 2 NADH, 2 Pyruvate | Net ATP:2 2 Pyruvate to 2 AcCoA: 2 NADH (6ATP) TCA Cycle: 6NADH (18) 2 FADH2 (4) 2GTP (2) 38 ATP Produced |
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Mitochondrial Genetics
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mtDNA codes for some mito proteins. These are synthesized by mitochondrial ribosomes which utilize mito tRNAs.
Codes complexes I, III, IV, and V. |
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Pathologies
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Leber's hereditary optic neuropathy: Blindless. Complex I protein mutation.
MELAS Syndrome: Myopathy, encephalopathy, lactic acidosis, stroke like episodes. Mitoleucine tRNA mutation. Congenital lactic acidosis: many different mutations, usually affecting complexes III and IV. |