Mitochondria B

Question 1

ATP synthesis is achieved by the ______ .

Answer 1

inner membrane embedded protein complex known as ATP synthase.

Question 2

ATP synthase consists from two main parts:.

Answer 2

F1 and F0

Question 3

F0 protein complex is located:

Answer 3

spannng the inner mitochondria membrane and forms a proton channel

Question 4

F1 protein complex is

Answer 4

bound to F0 and is an actual enzyme that makes ATP.

Question 5

F0 function

Answer 5

uses the energy of proton movement through the channel to generate ATP.

Question 6

How many protons are needed to generate 1 ATP?

Answer 6

3 protons

Question 7

Once made, ATP is transported out of mitochondria via ______

Answer 7

ATP-ADP antiporter

Question 8

mitochondria is also involved in regulating ______.

Answer 8

cell death

Question 9

Cell damage induces _______,

Answer 9

Bak/Bax-dependent permeabilization of outer mitochondria membrane

Question 10

Cytochrome c binds to several cytpolasmic proteins forming protein complex known as ______.

Answer 10

apoptosome

Question 11

Apoptosome activates ______

Answer 11

caspases, thus intiating apoptosis (regulated cell death).

Question 12

During ischemic injury, mitochondria also promotes ______

Answer 12

necrotic cell death.

Question 13

Ischemic injury results in ______

Answer 13

MPTP-dependent permeabilization of inner and outer mitochondria membranes, resulting in cytochrome release and elimination of proton gradient.

Question 14

_____ blocks ATP production.

Answer 14

lack of proton gradient

Question 15

In the absence of proton gradient, ATP synthase is converted into ______

Answer 15

ATPase, thus using up available ATP. That leads to ATP depletion and necrosis.

Question 16

release of Bak/Bax-dependent permeabilization of outer mitochondria membrane leads to _____

Answer 16

leads to cytochrome c release.

Question 17

Damaged mitochondria is not only uncapable of producing ____, but also generate excessive amounts of _____.

Answer 17

ATP

reactive oxygen (ROS)

Question 18

ROS causes _______

Answer 18

cell damage and senescence by oxidating various cellular proteins, lipids and DNA.

As the result, the mitochondria quality s strictly controlled at three levels.

Question 19

mitochondria control levels:

Answer 19

1. several mitochondrial proteases
2. mitophagy.
3. if mitochondria damage is extensive, mitochondria induces apoptotosis.

Question 20

mitochondrial proteases: examples and function?

Answer 20

1. mAAA
2. iAAA
3. Lon

responsible for recognizing and degrading mis-folded proteins.

Question 21

mitophagy

Answer 21

1. a way of fixing damaged mitochondria by fusing the damaged mitochondria with healthy mitochondria,
2. can also eliminate damaged mitochondria

Question 22

Accumulation of mitochondria damage and increase in ROS is related to _______.

Answer 22

senescence and increased sensitivity to neuronal degeneration

Question 23

In addition, several mutations of the proteins in mitochondria quality control pathways result in _____.

Answer 23

various neuropathys

Question 24

Mutations in mitochondria fusion machinery causes _____

Answer 24

1. autosomal dominant optic atrophy (OPA1 gene)

2. Charcot-Marie-Tooth neuropathy type 2A (Mfn2 gene).

Question 25

autosomal dominant optic atrophy gene?

Answer 25

OPA1 gene

Question 26

Charcot-Marie-Tooth neuropathy type 2A gene?

Answer 26

Mfn2 gene

Question 27

Mutation in mAAA protease causes _______.

Answer 27

hereditary spastic paraplegia

Question 28

arsenic toxin works by:

Answer 28

inhibiting oxidative phosphorylation and inhibiting ATP production.

Question 29

what mutation causes autosomal dominant optic atophy?

Answer 29

mitchondrial fusion

Question 30

what mutation causes heteditary spastic paraplegia?

Answer 30

mAAA protease

Question 31

what mutation causes carchot marie tooth neuropathy?

Answer 31

mitochondrial fusion

Question 32

mitochondria functions:

Answer 32

1. generate ATP
2. apoptosis
3. regulation of intracellular Ca ion

Question 33

mutations in Mfn and OPA cause

Answer 33

1. autosomal dominant optic atrophy

2. Charcot Marie Tooth neuropathy 2A

Question 34

role of mitochondria in cell death

Answer 34

1. regulation of cytosolic calcium
2. generation of ROS
3. supply energy
4. release of cytochrome c and activation of apoptosome