Ammonia

• excess are broken down

• generic way aa metabolized

• toxic to brain

• amino group passed to a keto to become glutamate

Glutamine

Alanine

• amino group passed to pyruvate to create alanine

• alanine taken to liver but can't be metabolized

Urea

• only in liver

• high levels of glutamate and acetyl coa in fed state

• activate urea cycle

• all area amino acids

• urea cycle happen in liver

• if no aa ingested, little aa breakdown

• only used in children

• arginine becomes essential

OTC Deficiency

Citrullinemia