Myoclonic Epilepsy w/ Ragged-Red Fibers (Mitochondrion)
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How many layers make up mito membrane?
2; Outer Mito Membrane is rich in porin (allows significant free-passage from cytoplasm) Inner- Unique; forms shelf-like folds called cristae;
T or F: # of Christae = ability to produce ATP; IMM contains proteins of ETS; Cardiolipin (found in abundance) decreases permeability of membrane to small ions (H+)
True true and true
What separates the IMM from the OMM (inner from the outer)?
Intermembrane space. H+ accumulation area
What fills the interior of Mito? (inside IMM)
A matrix; contains enzymes of Kreb’s cycle, nucleic acid machinery
What is the name of the theory for how a mito completes oxidative phosphorylation?
Chemiosmotic Coupling Hypothesis
Where does glycolysis occur?
In the cytoplasm conversion of glucose to pyruvate; pyruvate enter matrix and is converted to acetyl CoA
Where does Krebs (citric acid cycle) occur?
In the matrix Acetyl CoA enters a series of reactions and produces CO2, electrons, and protons; electrons and protons received mostly by NAD
Where does ETS happen?
On/in the Inner Mito Membrane (IMM) NADH releases H+ and e-; H+ pumped across IMM to intermembrane space; e- pulled along and through IMM by way of many IMPs; Separation of charges creates potential energy (-160mV inside edge; called proton-motive force) and matrix pH=8
Where does the majority of “action” happen during oxidative phosphorylation? (chemiosmotic coupling hypothesis)
On/In the IMM
What maintains the electrochemical gradient on each side of IMM?
A phospholipid called Cardiolipin
What is the name of the enzyme that allows for special re-entry in the IMM?
ATP synthetase (sometimes called "F1 particle" Allows H+ to re-enter the matrix as it converts ADP to ATP
Which direction does O2 move? Which direction does CO2 move? Which direction does ADP move? Which direction does ATP move?
O2 moves in b/c it is disappearing CO2 moves out ADP moves in ATP moves out
There are 5 major complexes of proteins within the IMM that make up the ETS. What are complexes 1 and 5?
1 = NADH dehydrogenase 5 = ATP synthetase
What drives the production of ATP?
controlled re-entry of H+ into the matrix
Which charge is on the outer edge of the IMM? Which charge is on the inside edge?
+ outside; - inside
Brown fat cells have “leaky” IMMs. What is the result?
Little ATP production and a lot of heat
What type of cells are most affected by Myoclonic Epilepsy w/ Ragged-Red Fibers?
Type 1 skeletal muscle cells; these cells are very dependent on high complement of mito
T or F: Only a few of the affected tissues contain damaged mitochondria?
False; All affected tissues contain damaged mito (gives ragged-red quality)
Myoclonic epilepsy w/ ragged-red fibers results from a failure of …?
Mitochondria. They lack critical electron transport system protein assemblies due to a mutation in mtDNA. Any cell that gets these mito AND has a substantial need for ATP will suffer.