Aromatic AA Flashcards
Nitrogen Balance & Inborn errors of metabolism
during periods of growth, pregnancy/lactation, or recovery from metabolic/surgical stress, nitrogen balance is said to be ?
positive
when there is an inadequate amount of protein, lack of essential AAs, or increased metabolic stress, nitrogen balance is said to be ?
negative
inadequate protein intake in children… means “disease of the displaced child” in Ghana
Kwashiorkor
starvation– inadequate intake of protein and total calories
marasmus
in phenylalanine degradation/tyrosine synthesis, the OH group is added to phenylalanine by which enzyme?
phenylalanine hydroxylase
Tyrosine is not an essential AA, but it can be essential if there is an inadequate amount of _____ in the diet
Phe
which AA competes with and blocks Tyr + Trp from crossing the blood-brain barrier?
phenylalanine
toxic product of Phe/Tyr degradation that reacts w/ amino groups inhibiting important processes including DNA repair
succinylacetone
elevated levels of branched chain AAs and their corresponding a-keto acids induce ketoacidosis, neurological impairment, developmental delays, coma, and death. The name of this disease is what?
Maple Syrup Urine Disease
which compounds lead to the buildup of propionic acid (propionic acidemia) ?
- Val
- odd chain FA chains
- Met
- Iso
- Thr
(V-O-M-I-T)
the inhibition of both the glycine cleavage complex and carbomyl phosphate synthase is caused by elevated levels of ?
propionic acid
when breaking down BCAAs, if propionyl-CoA, methylmalonyl-CoA, or succinyl-CoA are formed, the product will be described as ?
glucogenic
when breaking down BCAAs, if acetyl-CoA or acetoacetate are formed, the product will be described as ?
ketogenic
what are the notable neurotransmitters and hormones derived from Trp?
- serotonin
- melatonin
- acetyl-serotonin
phenylketonuria (PKU) is the inability to break down which AA?
phenylalanine
