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15 Cards in this Set

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Describe the enzyme, cofactor and products of the transanimase reaction.
Enzyme: Aminotransferase
Cofactor: PLP (VITAMIN B6)
α-amino acid: α-keotglutarate
α-keto acid: Glutamate
Describe the role of glutamate dehydrogenase in donating NH3 for the urea cycle
glutamate dehydrogenase gives a free ammonia to alanine in the muscles and then takes that ammonia back in the liver.
List the reactions that form ammonia in the liver
1. Transamination from alanine: (PLP)
2. Oxidative deamination from glutamate (glutamate dehydrogenase)
3. From Glutamine (glutaminase)
Describe ammonia formation in the gut
Bacterial ureases form ammonia in the colon. In those with liver disease, this ammonia is not detoxified.
Describe Hyperammonemia Type 1
Deficiency: CPS I
Pathology: Hyerpammonemia, neurological
Treatment: sometimes responds to Arginine to stimulate the formation of NAG which might stimulate CPS1.
Describe Hyperammonemia Type 2
Deficiency: OTC (ornithine transcarbamoylase)
Pathology: Hyperammonemia, increased orotic acid in urine
Treatment: ?
Describe Citrullinemia
Deficiency: Argininosuccinate synthetase
Pathology: Hyperammonemia, ↑ citrulline levels
Treatment: MAY include arginine to enhance citrulline excretion and provide high levels of substrate
Describe Argininosuccinic aciduria
Deficiency: ASL (Argininosuccinate lyase)
Pathology: Hyperammonemia, ↑ arginino succinate levels
Treatment: arginine for excretion
Describe Hyperargininemia
Deficiency: Arginase
Pathology: ↑ arginine levels
Treatment: diet of essential AAs excluding arginine.
What are the treatments for general Hyperammonemia?
1. Dialysis
2. Benzoic acid - to help excrete glycine (1 N / molecule)
3. Diet (low protein/high carb)
4. Medication (a-keto acids)
5. Stress prevention
6. Liver transplantation
What role does benzoic acid fill in treating hyperammonemia?
It is an amino acid scavenger.
It forms Benzoyl CoA which bonds with glycine to form Hippuratic acid. It takes 1 Nitrogen with it when excreted.
What role does phenylbutyrate fill in treating hyperammonemia?
It undergoes beta-oxidation to form phenylacetate which degrades to phenylacetyl CoA which combines with glutamine to form phenylacetylglutamine which takes 2 nitrogens with it when excreted.
What role does argenine fill in treating hyperammonemia?
It stimulates the formation of NAG which MIGHT stimulate deficient CPSI.
Describe Acquired hyperammonemia
Cause: Liver disease, cirrhosis
Pathology: Portal blood enters the systemic circulation without going to the liver.
Treatment:
1. Low protein/high carb diet
2. Lactulose which bacteria can't digest in gut
3. Neomycin (antibiotic to kill gut bacteria making lactate)