nitrogen Flashcards
where does our nitrogen come from?
amino acids and nucleotides
what is the metabolism of dietary proteins in the stomach?
Pepsin cuts protein into peptides
what is the metabolism of dietary proteins in the small intestine?
trypsin and Chymotrypsin cut proteins and large peptides into smaller peptides
- Aminopeptidase and Carboxypeptidases A and B degrade peptides into amino acids
what happens to all proteins?
All proteins are turned over and hence there is a need to replace them.
Some are highly stable lasting for days or weeks, while others may last for minutes.
what is the product of the degradation of cellular proteins?
amino acids
what type of cellular proteins get degraded?
Misfolded proteins
Foreign proteins
Unwanted proteins
describe the formation of urea
Amino acids come from:
- Ingested protein
- Alanine from muscle
- Glutamine from muscle and other tissues
Amino Acid metabolism produces ammonia NH4+
Ammonia goes in the Urea Cycle → Urea/Uric Acid out
what is phenylketonuria?
it’s when there is an absence/deficiency in Phenylalanine hydroxylase so the amino acid Phenylalanine can’t be converted to Tyrosine.
If not treated Phenylalanine → Phenylketones accumulate and can cause irreversible brain damage.
Treated by supplementing low protein diet with Tyrosine.
what is the most common urea cycle disorder?
ornithine transcarbamoylase (OTC)
describe hyperammonaemia:
elevated blood ammonia levels - medical emergency
highly toxic
They are typically present in the neonatal period.