Myoclonic Epilepsy w/ Ragged-Red Fibers (Mitochondrion) Flashcards

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1
Q

How many layers make up mito membrane?

A

2; Outer Mito Membrane is rich in porin (allows significant free-passage from cytoplasm)
Inner- Unique; forms shelf-like folds called cristae;

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2
Q

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+)

A

True true and true

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3
Q

What separates the IMM from the OMM (inner from the outer)?

A

Intermembrane space. H+ accumulation area

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4
Q

What fills the interior of Mito? (inside IMM)

A

A matrix; contains enzymes of Kreb’s cycle, nucleic acid machinery

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5
Q

What is the name of the theory for how a mito completes oxidative phosphorylation?

A

Chemiosmotic Coupling Hypothesis

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6
Q

Where does glycolysis occur?

A

In the cytoplasm

conversion of glucose to pyruvate; pyruvate enter matrix and is converted to acetyl CoA

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7
Q

Where does Krebs (citric acid cycle) occur?

A

In the matrix
Acetyl CoA enters a series of reactions and produces CO2, electrons, and protons; electrons and protons received mostly by NAD

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8
Q

Where does ETS happen?

A

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

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9
Q

Where does the majority of “action” happen during oxidative phosphorylation? (chemiosmotic coupling hypothesis)

A

On/In the IMM

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10
Q

What maintains the electrochemical gradient on each side of IMM?

A

A phospholipid called Cardiolipin

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11
Q

What is the name of the enzyme that allows for special re-entry in the IMM?

A
ATP synthetase (sometimes called "F1 particle" 
Allows H+ to re-enter the matrix as it converts ADP to ATP
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12
Q

Which direction does O2 move?
Which direction does CO2 move?
Which direction does ADP move?
Which direction does ATP move?

A

O2 moves in b/c it is disappearing
CO2 moves out
ADP moves in
ATP moves out

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13
Q

There are 5 major complexes of proteins within the IMM that make up the ETS. What are complexes 1 and 5?

A
1 = NADH dehydrogenase
5 = ATP synthetase
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14
Q

What drives the production of ATP?

A

controlled re-entry of H+ into the matrix

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15
Q

Which charge is on the outer edge of the IMM? Which charge is on the inside edge?

A

+ outside; - inside

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16
Q

Brown fat cells have “leaky” IMMs. What is the result?

A

Little ATP production and a lot of heat

17
Q

What type of cells are most affected by Myoclonic Epilepsy w/ Ragged-Red Fibers?

A

Type 1 skeletal muscle cells; these cells are very dependent on high complement of mito

18
Q

T or F: Only a few of the affected tissues contain damaged mitochondria?

A

False; All affected tissues contain damaged mito (gives ragged-red quality)

19
Q

Myoclonic epilepsy w/ ragged-red fibers results from a failure of …?

A

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.