Glycolysis and PDH

Question 1

Fructokinase deficiency yields

Answer 1

Nothing it is benign

Question 2

Galactokinase deficiency yields

Answer 2

Cataracts early in life

Question 3

Sucrase cleaves Sucrose to

Answer 3

Glucose and Fructose

Question 4

Cofactors and coenzymes for PDH

Answer 4

1. Thiamine phyrophasphate (Thiamine)
2. Lipoic acid
3. Coenzyme A (Pantothenate)
4. FAD(H₂) (Riboflavin)
5. NAD(H) (Niacin)

Question 5

Pyruvate kinase activated by

Answer 5

Fructose 1,6-BisP from the PFK-1 reaction

Question 6

Fructokinase converts

Answer 6

Fructose to Fructose 1-P

Question 7

Galactosemia genetics

Answer 7

AR

Question 8

Hexokinase/Glucokinase convert

Answer 8

Glucose to Glucose-6P

Question 9

Gal 1-P uridyltransferase deficiency yields

Answer 9

1. Cataracts early in life
2. Vomiting, Diarrhea
3. Lethargy
4. Liver damage (hyperbilirubinemia)
5. Mental retardation

Question 10

Pyruvate Kinase converts

Answer 10

Phosphoenolypyruvate (PEP) to Pyruvate

Question 11

Pyruvate => Lactate yields

Answer 11

NADH => NAD

Question 12

Pyruvate Kinase requires

Answer 12

ADP => ATP

Question 13

Aldolase B converts

Answer 13

Fructose 1-P to:

1. DHAP
2. Glyceraldehyde

Question 14

PFK-1 converts

Answer 14

Fructose-6P to Fructose-1,6-Bis Phosphate

Question 15

Aldolase converts

Answer 15

Fructose-1,6-BP to:

1. Glyceraldehyde-3P
2. Dihydroxyacetone-P (DHAP)

Question 16

PFK-1 requires

Answer 16

ATP => ADP (energy)

Question 17

Galactokinase converts

Answer 17

Galactose to Galactose 1-P

Question 18

Pyruvate dehydrogenase converts

Answer 18

Pyruvate to Acetyl-CoA

Question 19

Lactase cleaves Lactose to

Answer 19

Glucose and galactose

Question 20

PFK-1 stimulated by

Answer 20

Fructose 2,6-BisP and AMP

Question 21

PFK-2 converts

Answer 21

Fructose-6P to Fructose-2,6-Bis Phosphate

Question 22

Gal 1-P uridyl transferase converts

Answer 22

Galactose 1-P to Glucose 1-P

Question 23

Isomerase converts

Answer 23

Glyceraldehyde-3P <=> DHAP

Question 24

Energy producing enzymes in Glycolysis

Answer 24

Phophoglycerate kinase (1,3-BP to 3-Phosphoglycerate)

Pyruvate Kinase

Question 25

Aldolase B deficiency yields

Answer 25

1. Lethargy, vomiting 2. Liver damage (**hyperbilirubinemia**) 3. Hypoglycemia 4. Hypercuricemia 5. Renal proximal tubule defect (**Fanconi**)

Question 26

PFK-2 stimulated by

Answer 26

Insulin

Question 27

Glycolysis is

Answer 27

A cytoplasmic pathway that converts glucose to **two pyruvates**

Question 28

Hexokinase/glucokinase requires

Answer 28

Mg Insulin ATP =\> ADP (**energy**)

Question 29

Pyruvate Kinase deficiency leads to

Answer 29

Hemolytic anemia Increased BPG No heinz bodies

Question 30

DHAP is used for

Answer 30

Triglyceride synthesis by liver and adipose tissue

Question 31

Irreversible enzymes of Glycolysis

Answer 31

1. Glucokinase/Hexokinase 2. PFK-1 3. Pyruvate kinase

Question 32

Hereditary Fructose Intolerance genetics

Answer 32

AR

Question 33

Maltase Cleaves **Maltose** to

Answer 33

2 Glucose