Biochemistry Memorization

Question 1

LSD: Hunter

Answer 1

Iduronate Sulfatase

Question 2

LSD: Hurler & ScheieA

Answer 2

a-L-Iduronidase

Question 3

LSD: I-Cell Disease

Answer 3

Multiple enzymes due to a defect in the M-6-P targeting system.

Question 4

LSD: Pompe

Answer 4

Acid a-glucosidase

Question 5

LSD: Mucolipidosis VII

Answer 5

B-Glucuronidase

Question 6

Function of cofactor?

CoA/AcetylCoA

Answer 6

Transfers acyl groups

Question 7

Function of cofactor? Where on PDH?

Thiamine Pyrophosphate

Answer 7

Decarboxylation and 2 carbon transfer. On E1.

Question 8

Function of cofactor? Where on PDH?

Lipoic Acid/Lipoamide

Answer 8

Acetyl transfer. On E2.

Question 9

Function of cofactor? What enzyme?

Biotin

Answer 9

Carboxylation. Pyruvate Carboxylase (gluconeogenesis).

Question 10

Function of cofactor? What pathway(s)?

NAD+/NADH

Answer 10

Electron transfer for OXIDATION.

Question 11

Function of cofactor?

NADPH/NADP+

Answer 11

Electron transfer for REDUCTION.

Question 12

Coenzyme Deficiencies

Niacin (Vitamin B3)

Answer 12

Dermatitis
Diarrhea
Dementia
(Three-Ds)

Also called pellagra.

Question 13

Coenzyme Deficiencies

Thiamine (Vitamin B1)

Answer 13

Ophthalmoplegia
Gait difficulties
Confusion

Beri Beri or Wernicke-Korsakoff

Question 14

Coenzyme Deficiencies

Riboflavin (Vitamin B2)

Answer 14

Cheilosis

Glossitis

Question 15

Coenzyme Deficiencies

Lipoate

Answer 15

Targeted by arsenic:

Pyruvate DH & a ketoglutarate dehydrogenases DH

Question 16

Toxins of glycolysis–what enzyme?

2-deoxyglucose

Answer 16

hexokinase

Question 17

Toxins of glycolysis–what enzyme?

arsenate

Answer 17

G3P-DH

Question 18

Toxins of glycolysis–what enzyme?

Fluoride

Answer 18

Enolase

Question 19

Glycogen Storage Disease

Type I

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 19

1. Von Gierke
2. Glucose-6-phosphatase
3. Liver and kidney
4. Amount increase, normal structure
5. Severe hypoglycemia, hepatomegaly

Question 20

Glycogen Storage Disease

Type II

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 20

1. Pompe
2. a-1,4-glucosidase
3. Everywhere
4. Increased amount, normal structure
5. Cardiorespiratory failure

Question 21

Glycogen Storage Disease

Type III

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 21

1. Cori
2. Debranching enzyme
3. Muscle, liver
4. Increased amount, short outer branches.
5. Mild hypoglycemia and hepatomegaly (Like Type VI, a mild Type I)

Question 22

Glycogen Storage Disease

Type IV

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 22

1. Anderson
2. Branching Enzyme
3. Liver
4. Normal amount, long outer branches
5. Hepatomegaly/fatal.

Question 23

Glycogen Storage Disease

Type V

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 23

1. McArdle
2. Phosphorylase
3. Muscle
4. Increased amount, normal structure
5. Exercise problems, cramps (Like Type VII)

Question 24

Glycogen Storage Disease

Type VI

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 24

1. Hers
2. Phosphorylase
3. Liver
4. Increased amount, normal structure
5. Mild hypoglycemia and hepatomegaly (Mild Type I)

Question 25

Glycogen Storage Disease

Type VII

1. Name
2. Enzyme
3. Location
4. Glycogen changes
5. Features

Answer 25

1. Tauri’s
2. PFK1
3. Muscle
4. Increased amount, normal structure
5. Exercise problems, cramps (like Type V)