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10 Cards in this Set

  • Front
  • Back
Respiratory Quotient
1.0 for glucose
.70 for palmitic acid

Protein is somewhere in between.
Energy Storage
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.
Mitochondrial Structure
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
Mitochondrial energy production:
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.
Electron Chain
Purpose: Converstion of electrical to chemical energy.
Location: IMM
NADH and FADH2 converted to energy via ATP.
Regulation: NADH, FADH2, and ADP: uncoupling proteins
Organization of the ETC
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.
Regulation of ETC:
Regulated by availability of NADH and FADH. ADP> Thermogenin and other uncoupling proteins. Toxins: aspirin, DNP, inhibitors.
ENERGY Balance Sheet
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
Mitochondrial Genetics
mtDNA codes for some mito proteins. These are synthesized by mitochondrial ribosomes which utilize mito tRNAs.

Codes complexes I, III, IV, and V.
Pathologies
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.