Amino Acid Metabolism Pt. II Flashcards Preview

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Flashcards in Amino Acid Metabolism Pt. II Deck (38):
1

 

 

 

Phenylalanine Degredation 

2

 

 

 

Alcaptonuria

3

 

 

 

Phenylketonuria

 

 

 

Defect Associated with Phe Metabolism 

4

 

 

 

Tyrosinemia Type I

 

 

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

5

 

 

 

Tyrosinemia Type II

 

 

 

Treatment of Type II just stops production of homogentisiic acid

6

 

 

 

Biopterin Cycle

7

 

 

 

Neurotransmitter Structures

8

 

 

 

Neurotransmitter Structures Pt. II

9

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

GABA

Acetylcholine

Epinephrine

Serotonin

Glycine

 

 

 

GABA

10

 

 

 

Homocysteinemia/uria Pathway 

11

 

 

 

Homocysteinemia/uria Features

12

 

 

 

Causes of Elevated Homocysteine 

13

 

 

 

What does Homocysteine Do?

14

 

 

 

Treatments for Elevated Homocysteine 

15

Which reaction requires both Folate and B12?

Succinate to Succinyl CoA

SAH to Homocysteine

Homocysteine to methionine 

Homocysteine to Cystathionine

 

 

 

Homocysteine to methionine 

16

 

 

 

Homocysteine and Neural Tube Defects

17

 

 

 

What about Vitamin B12

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 

 

 

 

Branched Chain Fatty Acids

19

 

 

 

B12 and Folate Deficiencies

20

 

 

 

Liver and B12 Deficiencies

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

 

 

 

Inability to make DNA  

22

 

 

 

Branch Chain Amino Acids

23

 

 

 

Branch Chained Amino Acids Pt. II

24

 

 

 

Branch Chained Amino Acids Pt. III 

25

 

 

 

Branch Chained Amino Acid Metabolism

26

 

 

 

Glycine Metabolic Disorders

27

 

 

 

Non-Ketotic Hyperglycinemia

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 

 

 

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

29

 

 

 

Biosynthesis of Heme

30

 

 

 

Synthesis of Gamma Aminolevulinic Acid

31

 

 

 

Lead Poisoning

32

 

 

 

Heme Synthesis Pt. II

33

 

 

 

Defects in Heme Biosynthesis 

34

 

 

 

Heme Degredation 

35

 

 

 

Regulatory Aspects of Amino Acid Degredation 

36

 

 

 

Role of Alanine in Muscle Amino Acid Degredation 

37

 

 

 

Amino Acid Flux Between Tissues

38

 

 

 

 

 

 

 

Maple Syrup Urine Disease. 

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