Q1. Mitochondrial diseases are often very severe because: (Select ALL that apply

Question

Q1.

Mitochondrial diseases are often very severe because:

(Select ALL that apply.)

1. mitochondria contain most of the DNA in the cell.

2. mitochondria have their own genome.

3. they affect the cell’s energy metabolism.

4. ATP produced by the mitochondria is important for normal cellular function.

Q2.

If one child has a mitochondrial disease, all of that child’s siblings ------------------ always also have the mitochondrial disease because each egg cell ------------ the same set of mitochondria.

Select ONE option for the FIRST blank:

1. will

2. will not

Select ONE option for the SECOND blank:

1. has

2. does not have

Q3.

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Q4.

Mitochondrial fission is important for healthy mitochondrial function. Mitochondrial fission allows for old, defective components from one mitochondrion to be replaced with new ones. Mitochondrial fission facilitates this replacement of old mitochondrial parts by isolating them in small membrane-bound vesicles that are then degraded by the cell.

What is the expected consequence for mitochondrial energy production in a pathological condition where mitochondrial fission is reduced?

Select ONE option:

A. The rate of mitochondrial replacement will increase and ATP levels will increase.

B. The rate of mitochondrial replacement will decrease and ATP levels will decrease.

Q5.

The figure below shows fission (division) of one mitochondrion into two mitochondria. For this process to occur, the mitochondrion has to grow to a certain size by synthesizing the necessary components to increase its volume.

Part 1. In order for a mitochondrion to divide, it must first make more membrane. What are the major components that need to be made by the mitochondrion during this first step?

(Select ALL that apply.)

A. Outer membrane lipids

B. ATP synthase

C. Inner membrane lipids

D. Mitochondrial DNA

E. Cardiolipin

F. Pyruvate

G. Drp1

Part 2: Where is this type of enzyme likely to be located in the mitochondria?

Select ONE option:

A. Matrix

B. Inner mitochondrial membrane

C. Intermembrane space

D. Outer mitochondrial membrane

Q6.

Porins are integral membrane proteins that serve as channels for water molecules to go through a lipid bilayer. An example of a porin is shown below. The grey spheres indicate the phosphate heads of the phospholipid bilayer in which the porin is embedded. The cyan spheres indicate water molecules.

Part 1. Identify the region (A, B, or C), where you would expect to find the hydrophobic tails of the phospholipid bilayer.

Select ONE option:

1. A

2. B

Part 2. Given the images above, the red regions of the protein are most likely to be ____________.

Select ONE option for the blank:

1. hydrophobic

2. hydrophilic

Q7.

Transport along a microtubule is especially important in delivering mitochondria from the cell body of a motor neuron to the end of an axon, where there is a high energy demand for neurotransmission. Miro is an adaptor protein that binds mitochondria to the motor proteins responsible for carrying mitochondria along microtubules.

Part 1. What symptom(s) would you expect to see in a patient carrying a deletion to the miro gene?

(Select ALL that apply.)

1. Difficulty walking because the sciatic nerve, which is the longest cell in the body, will be impaired.

2. Vision loss due to impairment of short retinal neurons.

3. Deficiencies in neurons across entire nervous system, regardless of size.

Part 2. Disrupting which of the following processes would result in similar symptoms?

(Select ALL that apply.)

1. Microtubule organization

2. Motor protein interaction with microtubules

3. Actin filament oligomerization

Q8.

Recall that the inside of a phospholipid bilayer is hydrophobic, such that hydrophilic molecules cannot readily diffuse through a cell membrane. Which of the following would require a protein channel to cross a cell membrane?

(Select ALL that apply.)

1. chloride ions (Cl-)

2. sugar molecules

3. oxygen (O2)

4. amino acids

Q9.

Part 2. What will happen to an ATP molecule bound to a beta subunit in the T (tight) conformation when the gamma subunit rotates and causes the beta subunit to adopt the O (open) conformation?

Select ONE option:

1. The ATP molecule will be released.

2. The ATP molecule will be broken down to ADP and inorganic phosphate. incorrect

3. Nothing happens to either molecule yet.

Q10.

A single ATP synthase is capable of generating about 30 ATP molecules for every 100 protons that flow through its F0 complex. If we isolated a mutant ATP synthase in which one of its three beta-subunits could not bind ADP, how many ATP molecules would this mutant be expected to generate for every 100 protons that flow through it?

Select ONE option:

1. 30 ATP molecules

2. 20 ATP molecules

3. 10 ATP molecules

4. 0 ATP molecules

Standard IVF 3-Way IVF Location of fertilization

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. inside the body

B. outside the body

Number of genetic parents

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. 1

B. 2

C. 3

Source of mitochondrial DNA

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Source of nuclear DNA

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Drp 1 o

Explanation / Answer

Q1. 3. they affect the cell’s energy metabolism.

4. ATP produced by the mitochondria is important for normal cellular function.

Q2. If one child has a mitochondrial disease, all of that child’s siblings will always also have the mitochondrial disease because each egg cell has the same set of mitochondria. This is because the mitochondria is acquired by the mother always throught the mothers' (oocyte) cytoplasm and not by the father.

Q3.

Q3.

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Q4.

B. The rate of mitochondrial replacement will decrease and ATP levels will decrease.

.This is because the old and weary mitochondria will not be as effective in producing ATP as would the new and young mitochondria

Q5. Part 1:

A. Outer membrane lipids

B. ATP synthase

C. Inner membrane lipids

E. Cardiolipin

G. Drp1

Cardiolipin is a component of inner mitochondrial lipid constituents. ATP synthase is present in the inner membrane, Drp1 is a dynamin protein that assist in the fission process. Outer and inner lipid proteins are necessary to be made.

Q6.

2. B, Hydrophobic region is the middle portion which embedded in the lipid bilayer. region B

Red regions are hydrophobic. however the residues that face the pore are hydrophillic. the ones that face the lipid membran i.e appear red in the figure are hydrophobic.

Q7. Part 1: 3. Deficiencies in neurons across entire nervous system, regardless of size.

Part2: 1. Microtubule organization

2. Motor protein interaction with microtubules

Q8.

1. chloride ions (Cl-)

4. amino acids (charger side chains)

Sugar molecules are hydrophobic, oxygen is neutral an can diffuse easily depending on the size.

Q9.

1. The ATP molecule will be released.

Q10.

4. 0 ATP molecules

Standard IVF 3-Way IVF Location of fertilization

Select ONE option:

A. inside the body

B. outside the body

Select ONE option:

A. inside the body

B. outside the body

Number of genetic parents

Select ONE option:

A. 1

B. 2

C. 3

Select ONE option:

A. 1

B. 2

C. 3

Source of mitochondrial DNA

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Select ONE option:

A. Mt donor

B. Intended father

C. Intended mother

Source of nuclear DNA

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

Select ONE option:

A. Mt donor + intended father

B. Intended father + intended mother

C. mt donor

D. intended father

E. intended mother

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