Memetabolsim of nuclotides-pyrimidne and purine Flashcards
what are the atoms of pruine ring contributed by
aas (asparatate, glycine and glutamine)
CO2
n10-formyltetrahydrofolate
where is the purine ring constructed
in liver by series that add the carbons and nitrogens to ribose-5-phospgate
act and inhi of PRPP SYNTHASE
act: inorganic phosphate
inhi: pruine nuclotides
act and inhi GPAT
inihi: amp and gmp
act: controlled by intracellular concentration of PRPP
what inginit adenosine and guanosine monophospahte synthesis
by end product of that pathway
inhibitor of imp dehydrogenase and adenylosuccinate synthetase
revrsible inhibitor, mycophenolic acid
what is lesch nyhan syndrome
- xlinked disorder
deficiency of HGPRT –>result in inability to salvage hypoxanthine of G form which excessive amounts of uric acid is produced.
+ cause increased PRPP lvl and decreased IMP and GMP levels
what is regulative step in purine synthesis
GPAT
what is lesch nyhan a cause of and what does it resykt form
. The combination of decreased purine reutilization
and increased purine synthesis results in increased
degradation of purines and the production of large amounts
of uric acid, making HGPRT deficiency an inherited cause of
hyperuricemia
rsult in formation of uric acid stones in kidneys adn urate crustals in joints and soft tissues.
characteri: motor dysfunction, cognitive deficits, behavioral disturbances
what inhibit and act ribonucleotide reductase
inhi: dATP, hydroxyurea
act: ATP
where does purine nucleotide degradation occur
small intestine, where pancreatic nucleases hydrolyze them to nuclotides
what is gout initiated by + treatment
high levels of uric acid in blood (hyperuricemia) as result of either overproduction or underexcretion of uric acid
treatment: allopurinol inhibits xanthine oxidase, resulting in accumulation of hypoxanthine
what can hyperuricemia lead to
monosodium uraye crystals in joints
inflammatory response to cyrstals
uric acid stones in kidney
hyperuricemia-result fo
- decreased salvage of hypoxanthine and G and the subsequent increased availability of PRPP
- consequence of increased availability of purines
unrelated metabolic diseases, such as von Gierke disease or hereditary fructose
intolerance
what is the regualting step in pyrimidine synthesis
ihibiton and act
mammalian cellS: Synthesis of carbamoyl phosphate form GLUTAMINE AND CO2, catalyzed by CPSII.
inhi: UTP
act: PRPP