PBIO

Question 1

Type 0:

A. GSD 0
B. Andersen disease
C. Tarui disease

Answer 1

A. GSD 0

Question 2

Type 1:

A. Forbes-Cori disease
B. Hers disease
C. Von Gierke

Answer 2

C. Von Gierke

Question 3

Type 2:

A. Forbes-Cori disease
(rare)
B. Pompe disease
(serios type of GSD)
C. Andersen disease

Answer 3

B. Pompe disease
(serios type of GSD)

Question 4

Type 3:

A. Forbes-Cori disease
(rare)
B. McArdle disease
C. Andersen disease

Answer 4

A. Forbes-Cori disease
(rare)

Question 5

Type 4:

A. Tarui disease
B. McArdle disease
C. Andersen disease

Answer 5

C. Andersen disease

Question 6

Type 5:

A. GSD 0
B. Forbes-Cori disease
(rare)
C. McArdle disease

Answer 6

C. McArdle disease

Question 7

Type 6

A. GSD 0
B. Hers disease
C. McArdle disease

Answer 7

B. Hers disease

Question 8

Type 7

A. Pompe disease
B. Von Glerke
C. Tarui disease

Answer 8

C. Tarui disease

Question 9

Deficiency of GSD 0

A. Glycogen synthase
B. Myophosphorylase
C. Debranching enzyme

Answer 9

A. Glycogen synthase

Question 10

Deficiency of Von Gierke

A. Glycogen synthase
B. Myophosphorylase
C. Glucose 6-phosphate

Answer 10

C. Glucose 6-phosphate

Question 11

Deficiency of Pompe disease

A. Phosphprylase (necessary to
breakdown glycogen)
B. Acid maltase enzyme
C. Transglucosidase (glucosyl
transferase enzyme)

Answer 11

B. Acid maltase enzyme

Question 12

Deficiency of Forbes Cori disease

A. Debranching enzyme
B. Acid maltase enzyme
C. Transglucosidase (glucosyl
transferase enzyme)

Answer 12

A. Debranching enzyme

Question 13

Deficiency of Andersen disease

A. Debranching enzyme
B. Myophosphorylase
C. Transglucosidase (glucosyl
transferase enzyme)

Answer 13

C. Transglucosidase (glucosyl
transferase enzyme)

Question 14

Deficiency of McArdle disease

A. Phosphofructokinase
B. Myophosphorylase
C. Phosphprylase (necessary to
breakdown glycogen)

Answer 14

B. Myophosphorylase

Question 15

Deficiency of Hers diseases

A. Phosphofructokinase
B. Myophosphorylase
C. Phosphprylase (necessary to
breakdown glycogen)

Answer 15

C. Phosphprylase (necessary to
breakdown glycogen)

Question 16

Deficiency of Tarui disease

A. phosphofructokinase
B. Acid maltase enzyme
C. Glycogen synthase

Answer 16

A. phosphofructokinase

Question 17

Clinical features of GSD 0

A. Muscle damage
B. Muscle cramps
D. Liver damage

Answer 17

B. Muscle cramps

Question 18

Clinical features of Von Gierke

A. Liver damage
B. Cardiac damage
C. Muscle damage

Answer 18

A. Liver damage

Question 19

Clinical features of Pompe disease

A. Muscle cramps
B. Liver damage
C. Cardiac damage

Answer 19

C. Cardiac damage

Question 20

Clinical features of Forbes Cori disease

A. Muscle damage
B. Cardiac damage
C. Liver damage

Answer 20

C. Liver damage

Question 21

Clinical features of Andersen disease

A. Liver damage
B. Muscle crams
C. SOB

Answer 21

A. Liver damage

Question 22

Clinical features of McArdle disease

A. Liver damage
B. Muscle damage
C. Muscle cramps

Answer 22

B. Muscle damage

Question 23

Clinical features of Hers disease

A. Muscle cramps
B. Muscle damage
C. Liver damage

Answer 23

C. Liver damage

Question 24

Clinical features of Tarui disease

A. Cardiac damage
B. Muscle damage
C. Liver damage

Answer 24

B. Muscle damage

Question 25

Manifestation of GSD 0 A. Fasting hypoglycemia B. Extreme enlargement of liver (accumulation of glycogen) C. Muscle fatigue, Muscle pain

Answer 25

A. Fasting hypoglycemia

Question 26

Manifestation of Von Gierke A. Fasting hypoglycemia B. Exercise induce cramps C. Hepatomegaly, platelet dysfunction and growth retardation

Answer 26

C. Hepatomegaly, platelet dysfunction and growth retardation

Question 27

Manifestation of Pompe disease A. IIA- infantile form, IIB- juvenile, IIC- adult B. Lysosomal storage disease (accumulation of glycogen in lysosome) C. General: skeletal muscle weakness D. All the above

Answer 27

D. All the above

Question 28

Manifestation of Forbes Cori disease (rare) I. Hepatomegaly, hypoglycemia and myopathy II. Can lead to death within 5 years of age III. Affects liver, heart, skeletal muscle A. I only B. II only C. I and III

Answer 28

C. I and III

Question 29

Manifestation of Andersen Disease I. Rare inherited condition that causes severe muscle pain and cramps II. Abnormal glycogen in liver and muscle III. Can lead to death within 5 years of age A. I and II B. II and III C. II only

Answer 29

B. II and III

Question 30

Manifestation of McArdle disease I. Rare inherited condition that causes severe muscle pain and cramps II. Exercise induce cramps III. Rhabdomyolysis A. I only B. II and III C. I, II, and III

Answer 30

C. I, II, and III

Question 31

Manifestation of Hers disease I. Lysosomal storage disease (accumulation of glycogen in lysosome) II. Extreme enlargement of liver (accumulation of glycogen) III. Abnormal glycogen in liver and muscle A. I only B. II only C. I, II, and III

Answer 31

B. II only

Question 32

Manifestation of Tarui disease A. Muscle fatigue and Muscle pain B. Fasting hypoglycemia C. Hepatomegaly, hypoglycemia and myopathy

Answer 32

A. Muscle fatigue and Muscle pain

Question 33

Site of Cyanide A. Cytochrome oxidase B. ADP phosphorylation C. NADH-CoQ reductase

Answer 33

A. Cytochrome oxidase

Question 34

Effect of Cyanide A. All intermediates before and including cyt a will be in the reduced state; all intermediates after and including cyt c1 will be oxidized states. Blocks site lll B. Blocks transfer of electrons to O2. Blocks at site IV C. Inhibits entry of ADP into mitochondria and ATP export. Stop electron transport because of lack of ADP. Inside, all ADP is converted to ATP

Answer 34

B. Blocks transfer of electrons to O2. Blocks at site IV

Question 35

Site of Antimycin A. ADP phosphorylation B. ADP-ATP transporter C. Electron transfer from cyt b to cyt c1

Answer 35

C. Electron transfer from cyt b to cyt c1

Question 36

Effect of Antimycin A. All intermediates before and including cyt a will be in the reduced state; all intermediates after and including cyt c1 will be oxidized B. Blocks phosphorylation of ADP. Does not inhibit uncouples oxidations C. Blocks oxidation of NADH (site l). NADH will be reduced; Substrates such as succinate that enter via FADH will still be oxidized and make 2 ATPs/mol

Answer 36

A. All intermediates before and including cyt a will be in the reduced state; all intermediates after and including cyt c1 will be oxidized

Question 37

Site of Rotenone A. Cytochrome oxidase B. NADH-CoQ Reductase C. Electron transfer from cyt b to cyt c1

Answer 37

B. NADH-Co2 Redutase

Question 38

Effect of Rotenone A. Blocks transfer of electrons to O2. Blocks at site IV B. Blocks phosphorylation of ADP. Does not inhibit uncouples oxidations C. Blocks oxidation of NADH (site l). NADH will be reduced; Substrates such as succinate that enter via FADH will still be oxidized and make 2 ATPs/mol

Answer 38

C. Blocks oxidation of NADH (site l). NADH will be reduced; Substrates such as succinate that enter via FADH will still be oxidized and make 2 ATPs/mol

Question 39

Site of Oligomycin A. ADP Phosphorylation B. NADH-CoQ Reductase C. Cytochrome oxidase

Answer 39

A. ADP phosphorylation

Question 40

Effect of Oligomycin A. Blocks transfer of electrons to O2. Blocks at site IV B. Inhibits entry of ADP into mitochondria and ATP export. Stop electron transport because of lack of ADP. Inside, all ADP is converted to ATP C. Blocks phosphorylation of ADP. Does not inhibit uncouples oxidations

Answer 40

C. Blocks phosphorylation of ADP. Does not inhibit uncouples oxidations

Question 41

Site of Atractyloside and bongkrekate A. NADH-CoQ Reductase B. Electron transfer from cyt b to cyt c1 C. ADP-ATP transporter

Answer 41

B. ADP-ATP transporter

Question 42

Effect of Atractyloside and bongkrekate A. Blocks phosphorylation of ADP. Does not inhibit uncouples oxidations B. Inhibits entry of ADP into mitochondria and ATP export. Stop electron transport because of lack of ADP. Inside, all ADP is converted to ATP C. All intermediates before and including cyt a will be in the reduced state; all intermediates after and including cyt c1 will be oxidized

Answer 42

B. Inhibits entry of ADP into mitochondria and ATP export. Stop electron transport because of lack of ADP. Inside, all ADP is converted to ATP