Biochemistry

Question 0

Rate limiting enzyme for Gluconeogenesis

Answer 0

Fructose-1,6- bisphosphatase

Question 1

Rate limiting enzyme for Glycolysis

Answer 1

PFK-1

Question 2

Rate limiting enzyme for Glycogenesis

Answer 2

Glycogen synthase

Question 3

Rate limiting enzyme for Glycogenolysis

Answer 3

Glycogen phosphorylase

Question 4

Glycogen phosphorylase deficiency

Answer 4

McArdle Dz

Question 5

Glucose-6-Phosphatase deficiency

Answer 5

von Gierke dz

Question 6

Lactic acidosis, hyperlipidemia, hyperuricemia

Answer 6

von Gierke dz

Question 7

a-1,6- glucosidase deficiency

Answer 7

Cori dz

Question 8

a-1,4- glucosidase deficiency

Answer 8

Pompe dz

Question 9

Incr glycogen in liver, severe fasting hypoglycemia

Answer 9

von Gierke dz

Question 10

Hepatomegaly, hypoglycemia, hyperlipidemia (normal kidneys, lactate, Uric acid)

Answer 10

Cori dz

Question 11

Painful muscle cramps, myoglobinuria w strenuous exercise

Answer 11

McArdle

Question 12

Severe hepatosplenomegaly, enlarged kidneys

Answer 12

von Gierke

Question 13

Rate limiting step of Pentose Phosphate pathway

Answer 13

G6P dehydrogenase

Question 14

Galactokinase deficiency

Answer 14

Galactokinase deficiency

Question 15

Aldolase B deficiency

Answer 15

Fructose intolerance

Question 16

Lactase deficiency

Answer 16

Lactose intolerance

Question 17

Galactose-1-P- uridyltransferase deficiency

Answer 17

Classic Galactosemia

Question 18

Fructokinase deficiency

Answer 18

Essential Fructosuria

Question 19

What is the rate limiting enzyme for ketone body synthesis

Answer 19

HMG-CoA synthase

Question 20

What is the rate limiting enzyme for cholesterol synthesis

Answer 20

HMG-CoA reductase

Question 21

Rate limiting enzyme for B-oxidation of fatty acids

Answer 21

Carnitine Acyl transferase

Question 22

What are the essential amino acids

Answer 22

Phenylalanine, Valine, Threonine, Tryptophan, Isoleucine, Methionine, Histidine, Leucine, Lysine

Question 23

a-ketoacid dehydrogenase B1 deficiency

Answer 23

Maple syrup urine disease

Question 24

Rate limiting step in Purine synthesis

Answer 24

Glutamate PRPP amidotransferase

Question 25

What is the rate limiting step in pyrimidine synthesis

Answer 25

Carbamoyl Phosphatase synthetase II

Question 26

What are the sources of carbons in the formation of purines

Answer 26

CO2, THF, Glycine, Glutamine, Aspartate

Question 27

What are the sources of carbons in the formation of pyrimidines

Answer 27

CO2, Aspartate, Glutamine

Question 28

What accounts for the negative charge of DNA

Answer 28

Phosphate group

Question 29

What accounts for the positive charge of histones

Answer 29

Arginine, Lysine

Question 30

HGPRT Deficiency

Answer 30

Lesch-Nyhan syndrome

Question 31

Treatment of Lesch-Nyhan syndrome

Answer 31

Allopurinol

Question 32

Headache causing condition caused by excess Vit A

Answer 32

Psuedotumor cerebri (incr. CSF pressure)

Question 33

Clinical findings of B6 deficiency

Answer 33

Same as riboflavin deficiency + convulsions, hyper-irritability and peripheral neuropathy

Question 34

Characteristics of Dry Beri Beri

Answer 34

- Nonspecific peripheral neuropathy with myelin degeneration - Toe-drop, wrist-drop, foot-drop - Muscle weakness, Hyporeflexia, areflexia

Question 35

Characteristics of Wet Beri Beri

Answer 35

- Peripheral vasodilation --> high output cardiac failure --> peripheral edema - Cardiomegaly

Question 36

Rate limiting enzyme of TCA Cycle

Answer 36

Isocitrate dehydrogenase

Question 37

Rate limiting enzyme of Urea cycle

Answer 37

CPS I

Question 38

What is the rate limiting enzyme in fatty acid synthesis

Answer 38

Acetyl-CoA carboxylase

Question 39

Amino acid derivatives of Phenylalanine

Answer 39

- Tyrosine (requires BH4) --> Thyroxine - Dopa (requires BH4) --> Melanin - Dopamine (requires B6) - Norepinephrine (requires Vit. C) - Epinephrine (requires SAM)

Question 40

Amino acid derivatives of Tryptophan

Answer 40

- Niacin (requires B6) --> NAD+/NADP+ | - Serotonin (requires BH4) --> Melatonin

Question 41

Amino acid derivatives of Histidine

Answer 41

Histamine; requires B6

Question 42

Amino acid derivatives of Glycine

Answer 42

-Porphyrin (requires B6) --> Heme

Question 43

Amino acid derivatives of Arginine

Answer 43

-Creatinine, Urea, NO

Question 44

Amino acid derivatives of Glutamate

Answer 44

- GABA (requires B6) | - Glutathione