Amino Acid Metabolism Pt. II

Question 1

Phenylalanine Degredation

Answer 1

Question 2

Alcaptonuria

Answer 2

Question 3

Phenylketonuria

Answer 3

Defect Associated with Phe Metabolism

Question 4

Tyrosinemia Type I

Answer 4

When Fumarylacetoacetate Hydrolase accumulates it gets reduced to Succinylacetoacete (diagnostic) which leads to kidney and liver damage.

Question 5

Tyrosinemia Type II

Answer 5

Treatment of Type II just stops production of homogentisiic acid

Question 6

Biopterin Cycle

Answer 6

Question 7

Neurotransmitter Structures

Answer 7

Question 8

Neurotransmitter Structures Pt. II

Answer 8

Question 9

An OTC Defect may interfere with the synthesis of which neurotransmitter in the brain?

GABA

Acetylcholine

Epinephrine

Serotonin

Glycine

Answer 9

GABA

Question 10

Homocysteinemia/uria Pathway

Answer 10

Question 11

Homocysteinemia/uria Features

Answer 11

Question 12

Causes of Elevated Homocysteine

Answer 12

Question 13

What does Homocysteine Do?

Answer 13

Question 14

Treatments for Elevated Homocysteine

Answer 14

Question 15

Which reaction requires both Folate and B12?

Succinate to Succinyl CoA

SAH to Homocysteine

Homocysteine to methionine

Homocysteine to Cystathionine

Answer 15

Homocysteine to methionine

Question 16

Homocysteine and Neural Tube Defects

Answer 16

Question 17

What about Vitamin B12

Answer 17

Question 18

The use of methylmalonic acid for fatty acid biosynthesis will lead to which one of the following?

Short Chain Fatty Acids

Odd Chain Fatty Acids

Branched Chain Fatty Acids

Very Long Chain Fatty Acids

No Fatty Acids will be Synthesized

Answer 18

Branched Chain Fatty Acids

Question 19

B12 and Folate Deficiencies

Answer 19

Question 20

Liver and B12 Deficiencies

Answer 20

Question 21

The classic finding in someone with b12 deficiency is megaloblastic anemia— large immature blood cells. Why?

Inability to make homocysteine

Inability to Make Succinyl CoA

Inability to make DNA

Inability to Make RNA

Answer 21

Inability to make DNA

Question 22

Branch Chain Amino Acids

Answer 22

Question 23

Branch Chained Amino Acids Pt. II

Answer 23

Question 24

Branch Chained Amino Acids Pt. III

Answer 24

Question 25

Branch Chained Amino Acid Metabolism

Answer 25

Question 26

Glycine Metabolic Disorders

Answer 26

Question 27

Non-Ketotic Hyperglycinemia

Answer 27

Question 28

Hyperglycemia may also result from which one of the following? PKU B12 Deficiency Vitamin C Deficiency B1 Deficeincy Using an MAO inhibitor OTC Deficiency

Answer 28

B12 Deficiency — Leads to functional folate deficiency and the formation of glycine its conversion to serine requires folate.

Question 29

Biosynthesis of Heme

Answer 29

Question 30

Synthesis of Gamma Aminolevulinic Acid

Answer 30

Question 31

Lead Poisoning

Answer 31

Question 32

Heme Synthesis Pt. II

Answer 32

Question 33

Defects in Heme Biosynthesis

Answer 33

Question 34

Heme Degredation

Answer 34

Question 35

Regulatory Aspects of Amino Acid Degredation

Answer 35

Question 36

Role of Alanine in Muscle Amino Acid Degredation

Answer 36

Question 37

Amino Acid Flux Between Tissues

Answer 37

Question 38

Answer 38

Maple Syrup Urine Disease.