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BMB 4450

Nitrogen Metabolism and Disorders

QuestionAnswer
After ingesting dietary protein, amino acids are taken up into what border? intestinal brush border
Name at least 3 essential amino acids: valine, leucine, isoleucine, phenylalanine, tryptophan, lysine, histidine, methionine, threosine
In the fed state, what are excess amino acids converted into? glucose (glycogen storage) or triacylglycerols
During the fasting state, protein breaks down into amino acids for energy production. What amino acids is nitrogen stored in for waste removal? glutamine and alanine
What organ will receive glutamine as a nitrogen storage? kidney
What is the function of transaminases/aminotranferases? to transfer nitrogen groups from one compound to another in order to form amino acids
What is deamination? the removal of an amine group
What is deamidation? the removal of an amide group
What is the difference between an amine group and an amide group? amine groups consists of a nitrogen bonded to two hydrogens; amide groups consist of a nitrogen bonded to a carbonyl group
Glucogenic amino acids can be converted into glucose through gluconeogenesis. What are some examples? alanine, glutamate, arginine
Ketogenic amino acids can be degraded into ketone bodies. What are some examples? phenylalanine, tyrosine, leucine
What amino acid is important in the synthesis and degradation of amino acids? glutamate
What amino acid is the second source of nitrogen in the urea cycle? aspartate
In the glucose-alanine cycle, glutamate and pyruvate in muscle cells go through a transaminase reaction, yielding what two products? alanine and a-ketoglutarate
What happens in the glutamine-glutamate cycle? glutamate is converted into glutamine which is then shuttled to the liver to enter the urea cycle
What organelle does the generation of carbamoyl phosphate and citrulline occur in the cell during the urea cycle? mitochondria
List the molecule steps to the urea cycle: carbamoyl phosphate -> citrulline -> ornithine transporter -> arginine -> ornithine + urea
What is the function of arginase? convert arginine into ornithine and urea
What molecule activates carbamoyl phosphate synthase I allosterically? N-acetylglutamate
Ammonia toxicity in brain cells can lead to that dysfunction/disease? encephalopathy
What is hyperammonemia (HA)? a metabolic disorder caused by elevated levels of ammonia
Phenylketonuria (PKU) is a genetic disorder that prevents the breakdown of what amino acid? phenylalanine
In phenylketonuria (PKU), phenylalanine cannot be broken down into which amino acid? tyrosine
What does nitric oxide synthase (NOS) do? convert arginine into nitric oxide
Created by: gracelisabethxo
 

 



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