Amino Acid Metabolism Pt. II Flashcards Preview

Fundamentals of Molecular Medicine > Amino Acid Metabolism Pt. II > Flashcards

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

 

 

 

Phenylalanine Degredation 

A image thumb
2

 

 

 

Alcaptonuria

A image thumb
3

 

 

 

Phenylketonuria

 

 

 

Defect Associated with Phe Metabolism 

A image thumb
4

 

 

 

Tyrosinemia Type I

 

 

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

A image thumb
5

 

 

 

Tyrosinemia Type II

 

 

 

Treatment of Type II just stops production of homogentisiic acid

A image thumb
6

 

 

 

Biopterin Cycle

A image thumb
7

 

 

 

Neurotransmitter Structures

A image thumb
8

 

 

 

Neurotransmitter Structures Pt. II

A image thumb
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 

A image thumb
11

 

 

 

Homocysteinemia/uria Features

A image thumb
12

 

 

 

Causes of Elevated Homocysteine 

A image thumb
13

 

 

 

What does Homocysteine Do?

A image thumb
14

 

 

 

Treatments for Elevated Homocysteine 

A image thumb
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

A image thumb
17

 

 

 

What about Vitamin B12

A image thumb
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

A image thumb
20

 

 

 

Liver and B12 Deficiencies

A image thumb
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

A image thumb
23

 

 

 

Branch Chained Amino Acids Pt. II

A image thumb
24

 

 

 

Branch Chained Amino Acids Pt. III 

A image thumb
25

 

 

 

Branch Chained Amino Acid Metabolism

A image thumb
26

 

 

 

Glycine Metabolic Disorders

A image thumb
27

 

 

 

Non-Ketotic Hyperglycinemia

A image thumb
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

A image thumb
30

 

 

 

Synthesis of Gamma Aminolevulinic Acid

A image thumb
31

 

 

 

Lead Poisoning

A image thumb
32

 

 

 

Heme Synthesis Pt. II

A image thumb
33

 

 

 

Defects in Heme Biosynthesis 

A image thumb
34

 

 

 

Heme Degredation 

A image thumb
35

 

 

 

Regulatory Aspects of Amino Acid Degredation 

A image thumb
36

 

 

 

Role of Alanine in Muscle Amino Acid Degredation 

A image thumb
37

 

 

 

Amino Acid Flux Between Tissues

A image thumb
38

 

 

 

 

Q image thumb

 

 

 

Maple Syrup Urine Disease. 

Decks in Fundamentals of Molecular Medicine Class (77):