Protein and Amino Acid Metabolism III Flashcards
what are teh ways the body generates intermiedates
TCA cycle
converted into glucose
converted into keton bodies
what are the amino acids that are purely ketogenic
leucine and lysine
what is protein indicative of
low energy charge - catabolic state of body
oxidative glutamate dehydrogenase reaction
glutamate goes into alphaketoglutatrate via glutamate dehdrogenase
what is glutamate dehydrogenase regualted by
cellular energy chagne
what inhibits glutamate dehydrogenase
GTP
NADH
what stimulates glutamate dehydrogenase
ADP
causes of familial hyperinsulinemia hypoglycemia type 6
mutant form of glutamate dehydrogenase that’s insensitive to inhibition by GTP
symptom of HHF6
hypoglycermia
hyperammonemia
what can be converted into pyruvate
alanine
serine
glycine
cysteinee
what causes kidney stones
calcium oxalate precipitating in kidney tubules
major route of glycine catabolism
mitochondrial glycine cleavage system
where is the mitochondrial glycine cleavage system active
liver
reaction of mitochondrial glycine cleaaver system
glycine + THF —-> N5, N10 methlene TNF
is the mitochondrial glycine cleavage system reversible
yes
glycine encephalophthy is caused by what
defect in glycine cleaavage system
symptoms of glycine encephalophaty
lethargy, lac, o fmuscle tone, msucle twitching, death
what can be used to make OAA
pyruvate, aspargine, asparate
how is aspargine converted into asparate
asparaginase
how is aspartate converted to OAA
AST
what can be converted into alpha ketoglutarate
glutamate, glutamine, proline, arginine, ornithine, histdine
what AA can be converted into propionyl CoA
isoleucine, threonine, methionine and valine
what does the convertion of L methylmalonyl COA to succinyul CoA require as a coenzyme
vit B12
what causes propionic acidemia
defect inpropinoyl CoA carboxylase
what casues D methylmalonic aciduria
racemase deefect
what causes methylmalonic acidura
defect in mutase of vit B12
convertion of propionyl CoA to D methlmalonyl CoA requires what
biotin
what is the ultimate source of vit B12
bacteria
what is the best dietary sources of B12
meat, shellfish
what does vit B12 bind to
intrinsic factor
where does absorption of vit B12 happen
ileum
once absorbed what does vit B12 bind to
transcobalamin
what casues pernicious anemia
lack of intrinsic factor
what is the only reaction that can convert N50methyl THF back to THF
conversion of homocysterine to methionine catalyzed by methionine synthase
what does the convertino of N5 methyl THF back to THF require
vit B12
what does vit B12 defiency result in
accumualtion of N5 mthyl THF
what is N5N10 methylene THF required for
synthesis of thymidine and purine rings
megaloblastic anemia
cells get super large because they can’t complete cell dvision
