1.1.2 Amino Acid Transport & Deamination Flashcards
Gamma-glutamyl cycle enzymes are needed for what?
- There enzymes constitute and amino acid transport system
- Involved in the synthesis of glutathione
- The production of of extracellular gamma glutamyl amino acids which can be taken into cells and converted into 5-oxoproline which stimulates the prevalent form of amino acid transport
Describe nitrogen balance and compare positive and negative nitrogen balance.
What is the importance of nitrogen balance and ammonia?
Ammonia is toxic to the CNS
What can be an outcome if there is a problem in the body’s ability to make urea or eliminate it through the kidneys?
What is the importance of benzoic acid and phenylacetate?
These two compounds offer alternative ways of detoxifying and excreting ammonia
How is the Urea cycle regulated in the short term and long term?
Glutamate dehydrogenase catalyzes the reaction of what?
Glutamate to alpha ketoglutarate
This is important
Carbamoyl phosphate combines with ornithine to create?
Citrulline - Mitochondrial processese
What are the uses for ammonia?
Deficiency in what enzyme leads to Type 2 Hyperammonemia?
What is the reaction carried out by carbamoyl phosphate synthetase and why is important about it?
What are some of the sources of ammonia?
Intestinal bacterial ureases and ammonia reabsorption
What enzyme deficiency will lead to hyperargininemia?
Citrulline will eventually be converted into arginine which is a precursor for what?
Urea
Deficiency in what enzyme will lead to argininosuccinic aciduria?
How are amino groups released from amino acids?
By oxidative reactions or transfer reactions
Deficiency in what two enzymes can lead to type one Hyperammonemia?
Deficiency in what enzyme can lead to citrullinemia?
Glutamate dehydrogenase and amino transferases are involved in?
The synthesis and degradation of amino acids
Arginine can be decarboxylated to form what? Why is this important?
It can be decarboxylated to form agmatine - It is an intermediate to forming some polyamines, but also apprears to have neurotransmitter capabilities
Glutamine synthetase and glutaminase are involved in?
The storage, production and transport of the equivalent of ammonia
