Test3- Diseases and cofactors

Question 1

Carboxlyases use what as a cofactor?

Answer 1

Biotin

Question 2

What does Methylmalonyl CoA mutase need as a cofactor

Answer 2

Deoxyadenosyl cobalamin (B12)

Question 3

What is the rate limiting enzyme of lipolysis

Answer 3

Hormone sensitive lipase

Question 4

Hormone sensitive lipase is ________ when it is on

Answer 4

Phosphorylated

Question 5

What is the rate limiting enzyme of glycolysis?

Answer 5

PFK-1

Question 6

What is hexokinase inhibited by

Answer 6

G6P (its product)

Question 7

What is glucokinase inhibited by?

Answer 7

Nothing, no product inhibition

Question 8

What is PNDM from?

Answer 8

Question 9

What pathway is active when PFK-2/FBP-2 is phosphorylated?

Answer 9

Gluconeogenesis

Question 10

PFK-2 is ________ when active

Answer 10

Dephosphorylated

Question 11

Pyruvate kinase is active when it is ______

Answer 11

Dephosphorylated

Question 12

PDHC is active when _______

Answer 12

Dephosphorylated

Question 13

What happens with a PDHC deficiency?

Answer 13

Question 14

What happens with a thiamine deficiency? What is the syndrome that can manifest?

Answer 14

Question 15

What is the rate limiting enzyme of TCA cycle?

Answer 15

Isocitrate dehydrogenase

Question 16

What happens with arsenic poisoning?

Answer 16

Question 17

What is LHON?

Answer 17

Question 18

What is Leigh syndrome? What is the presentation?

Answer 18

Question 19

What are the inhibitors of the ETC and what are the important examples and treatment?

Answer 19

Question 20

What are the examples of uncouplers of the ETC and the consequences? What are the natural uncouplers?

Answer 20

Question 21

What happens with hyperthyroidism?

Answer 21

Question 22

What happens with atractyloside poisoning?

Answer 22

Question 23

What is the rate limiting enzyme of glycogenesis?

Answer 23

Glycogen synthase

Question 24

Where does glycogenesis occur

Answer 24

Cytosol

Question 25

Where does Liver Glycogenolysis occur

Answer 25

Cytosol

Question 26

What is the rate limiting enzyme of Liver Glycogenolysis

Answer 26

Glycogen phosphorlyase

Question 27

What is the cofactor needed by glycogen phosphorylase?

Answer 27

Pyridoxal phosphate B6

Question 28

Where does gluconeogenesis occur

Answer 28

In the liver and kidney cortex, in mitochondrial matrix and cytosol

Question 29

What is the rate limiting enzyme of gluconeogenesis?

Answer 29

FBPase-1

Question 30

What happens with a biotin deficiency?

Answer 30

Question 31

What happens with a PEPCK deficiency?

Answer 31

Question 32

What is the rate limiting enzyme of the HMP shunt?

Answer 32

G6PD

Question 33

Where does the HMP shunt take place?

Answer 33

Cytosolic pathway in all cells

Question 34

What happens with a GALT deficiency?

Answer 34

Question 35

What happens with an aldolase B deficiency?

Answer 35

Question 36

What happens with low insulin levels? What does Niacin inhibit?

Answer 36

Question 37

What is the rate limiting enzyme for Beta oxidation?

Answer 37

Carnitine shuttle (CPT-I enzyme*)

Question 38

Where does Beta oxidation take place

Answer 38

Mitochondria

Question 39

What is the rate limiting enzyme of ketogenesis?

Answer 39

HMG-CoA synthase

Question 40

What happens with continuously low I/G ratio?

Answer 40

Question 41

What is Jamaican vomiting sickness?

Answer 41

Question 42

What is the rate limiting enzyme of Lipogenesis

Answer 42

Acetyl-CoA carboxylase

Question 43

Where does Lipogenesis occur

Answer 43

Liver, cytosol on surface of SER

Question 44

Decrease in bile salts can be from what

Answer 44

Cholestasis from liver or bile duct

Question 45

Decrease in pancreatic enzymes can be from what

Answer 45

Cystic fibrosis, pancreatitis, EPI

Question 46

Decreased lipid absorption can be from what

Answer 46

IBD