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

Amino Acid Metabolism Pt. II Flashcards

1
Q

Phenylalanine Degredation

A
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2
Q

Alcaptonuria

A
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3
Q

Phenylketonuria

A

Defect Associated with Phe Metabolism

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4
Q

Tyrosinemia Type I

A

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

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5
Q

Tyrosinemia Type II

A

Treatment of Type II just stops production of homogentisiic acid

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6
Q

Biopterin Cycle

A
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7
Q

Neurotransmitter Structures

A
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8
Q

Neurotransmitter Structures Pt. II

A
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9
Q

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

GABA

Acetylcholine

Epinephrine

Serotonin

Glycine

A

GABA

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10
Q

Homocysteinemia/uria Pathway

A
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11
Q

Homocysteinemia/uria Features

A
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12
Q

Causes of Elevated Homocysteine

A
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13
Q

What does Homocysteine Do?

A
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14
Q

Treatments for Elevated Homocysteine

A
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15
Q

Which reaction requires both Folate and B12?

Succinate to Succinyl CoA

SAH to Homocysteine

Homocysteine to methionine

Homocysteine to Cystathionine

A

Homocysteine to methionine

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16
Q

Homocysteine and Neural Tube Defects

A
17
Q

What about Vitamin B12

A
18
Q

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

A

Branched Chain Fatty Acids

19
Q

B12 and Folate Deficiencies

A
20
Q

Liver and B12 Deficiencies

A
21
Q

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

A

Inability to make DNA

22
Q

Branch Chain Amino Acids

A
23
Q

Branch Chained Amino Acids Pt. II

A
24
Q

Branch Chained Amino Acids Pt. III

A
25
Q

Branch Chained Amino Acid Metabolism

A
26
Q

Glycine Metabolic Disorders

A
27
Q

Non-Ketotic Hyperglycinemia

A
28
Q

Hyperglycemia may also result from which one of the following?

PKU

B12 Deficiency

Vitamin C Deficiency

B1 Deficeincy

Using an MAO inhibitor

OTC Deficiency

A

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

29
Q

Biosynthesis of Heme

A
30
Q

Synthesis of Gamma Aminolevulinic Acid

A
31
Q

Lead Poisoning

A
32
Q

Heme Synthesis Pt. II

A
33
Q

Defects in Heme Biosynthesis

A
34
Q

Heme Degredation

A
35
Q

Regulatory Aspects of Amino Acid Degredation

A
36
Q

Role of Alanine in Muscle Amino Acid Degredation

A
37
Q

Amino Acid Flux Between Tissues

A
38
Q
A

Maple Syrup Urine Disease.

Fundamentals of Molecular Medicine (77 decks)

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