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32 Cards in this Set

  • Front
  • Back
In the electron transport chain, what does it do overall in terms of reoxidizing?
-it reoxidizes the coenzymes reduced in glycolysis and the TCA cycle
What are the 4 complexes of the ETC?
1) NADH-Ubiquinone Oxidoreductase
2) Succinate - Ubiquinone Oxidoreductase
3) Ubiquinol - Cytochrome C Oxidoreductase
4) Cytochrom C Oxidase
How are electrons passed through Complex 1?
-elctrons from NADH to FMN
-then to a series of Fe-S proteins
-Lastly passed to Ubiquinone
How many electrons can NADH carry?
FMN?
Fe-S proteins?
Ubiquinone?
-2
-1 or 2
-1
-2
What are the two possible centers of Iron-Sulfate proteins?
-2FE-2S or 4Fe-4S complexes
What was the case of 'Living Statues"?
-Heroin users who used a certain synthetic heroin became frozen with advanced stages of Parkinsons Disease
-this was from MPTP in the synthetic being converted to MPP
-MPP attacked the substantia nigra degrading the cells rapidly
Describe MPP in the body.
-a powerful inhibitor of Complex I in the mitochonrial ETC
-attacks the substantia nigra part of the brain
-Parkinson's patients have the same deterioration, but much slower
What is dopamines role in Parkinson treatment?
-brain cells can no longer produce dopamine, so injections can be given to provide it
-it allows for smooth muscle control/movement. w/o it, muscles are rigid and hard to control
What is Complex II also known as?
-Succinate Dehydrogenase (from TCA cycle)
Describe what Complex II does?
-contains a covalentrly bound FAD and two Fe-S proteins
-also transfers electrons to Ubiquinone (like C-I)
Describe Complex III.
-passes electrons from reduced Ubiquinone to Cytochrome C
What are cytochromes?
-small membrane associated proteins with a heme
--different classes have different substitutions on the heme group
Spectromphotomically, how can you tell cytochromes apart?
-when reduced, they have different absorbancies
What does Complex IV do?
-transfers electron from Cytochrome C to Oxygen
-three largest proteins are coded for in the mitochondrial DNA
-these three proteins contain the copper ions and two iron cofactors (cytochromes a and a3)
How many electrons does the reduction of Oxygen require?
-4 electrons
*copper and heme iron can only handle 1 electron
What are the parts 1 and 2 (of 4) of the Chemiosmotic Hypothesis?
1) mitochondrial inner membrane (MIM) is impermeable to H+
2) MIM has carrier proteins to mediate entry/exit of metabolites
What is part 3 of the Chemiosmotic Hypothesis?
3) ETC is proton-translocating and pumps H+ out of matric space when electrons are transported
What is the 4th part of Chemiosmotic Hypothesis?
4) mitochondrial ATP synthase complex also translocates protons across membrane. It can use the energy of proton gradient to drive ATP synthsis
What are 4 peices of evidence for the Chemiosmotic theory?
- mit. membranes can establich proton gradient
- intact MIM is required for oxidative phosphorylation
- key electron transport proteins spand the inner membrane
- Uncouplers act by dissipating the proton gradient
In respiration of mitochondria, what two things are coupled?
-oxidation of substrates
-phosphorylation of ADP
What must be present for respiration to proceed in Mitochonrion?
-ADP must be present
*amount of O2 consumed depends upon the amount of ADP added
What does Uncoupler DNP do?
-allows for O2 consumption without ADP
Describe Uncouplers.
-allow for O2 consumption without ADP
-lipid-soulbe weak acids
-can cross MIM
-deplete any proton gradient by transporting protons across the membrane
Give an example of a common Uncoupler.
-2.4-Dinitrophenol
Why can both acidic and Basic forms of DNP dissolve in the membrane?
-becuase negative charge is delocalized over the ring
What is Mitochondrial Inner Membrane ATPase for?
-treatment on inside-out particles to give free F1
-Part remaining in membrane is F0
What does F1 do?
-hydrolizes ATP (can NOT synthesize ATP)
What does F0 do?
-catalyze proton transport (can NOT synthesize ATP)
What does F1F0 ATPase do?
-Haloenzyme that uses proton motive force to convert ADP back to ATP
What are two Mitochondrial diseases?
-Leber's Hereditary Optic Neurophothy (LHON)
-Mutations in Cytochrome b
Describe Hereditary Optic Neurophathy (LHON).
-causes sudden-onset blindness
-mutation in mitochondrial DNA for proteins in electron transport complex I
-can cause dystonia
Describe mutations of Cytochrome b.
-exercise intolerance, lactic acidosis in resting state and myoglobinuria
-somatic mutation in the gene for cytochrome B