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BMB 4450
Nitrogen Metabolism and Disorders
| Question | Answer |
|---|---|
| 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 |