Amino Acid Metabolism Pt. II Flashcards
Phenylalanine Degredation

Alcaptonuria

Phenylketonuria
Defect Associated with Phe Metabolism

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

Tyrosinemia Type II
Treatment of Type II just stops production of homogentisiic acid

Biopterin Cycle

Neurotransmitter Structures

Neurotransmitter Structures Pt. II

An OTC Defect may interfere with the synthesis of which neurotransmitter in the brain?
GABA
Acetylcholine
Epinephrine
Serotonin
Glycine
GABA
Homocysteinemia/uria Pathway

Homocysteinemia/uria Features

Causes of Elevated Homocysteine

What does Homocysteine Do?

Treatments for Elevated Homocysteine

Which reaction requires both Folate and B12?
Succinate to Succinyl CoA
SAH to Homocysteine
Homocysteine to methionine
Homocysteine to Cystathionine
Homocysteine to methionine
Homocysteine and Neural Tube Defects

What about Vitamin B12

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
B12 and Folate Deficiencies

Liver and B12 Deficiencies

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
Branch Chain Amino Acids

Branch Chained Amino Acids Pt. II

Branch Chained Amino Acids Pt. III

Branch Chained Amino Acid Metabolism

Glycine Metabolic Disorders

Non-Ketotic Hyperglycinemia

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.
Biosynthesis of Heme

Synthesis of Gamma Aminolevulinic Acid

Lead Poisoning

Heme Synthesis Pt. II

Defects in Heme Biosynthesis

Heme Degredation

Regulatory Aspects of Amino Acid Degredation

Role of Alanine in Muscle Amino Acid Degredation

Amino Acid Flux Between Tissues


Maple Syrup Urine Disease.