Biochem- Purines & Pyrimedines
Terms
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- pyrimadines
-
CUT
CUT the PY - purines
-
AG
PUR As Gold - PRPP synthetase
- enzyme in first step of pur & pyr synthesis, converts ribose-5 phosphate to PRPP
- more metabolic diseases associated with ______ metabolism
- purine
- hydroxyurea
- an antineoplastic agent that works on ribonucleotede reductase enzyme and inhibits pyrimidine synthesis
- drugs - methotrexate (chemo), trimethotrim (antibiotic), & pyramethamine act on what enzyme
- dihydrofolate reductase in pyrimadine synthesis
- 5 FU acts on ______ enzyme in the production of ________
- thymidylate synthase in pyrimadines synthesis
- Orotic Aciduria
- elevated blood levels of orotic acid with NO associated hyperammonemia
- Inosine
- hypoxanthine + Ribose
- PRPP Amidotransferase
- important enzyme in purine synthesis inhibited by AMP, GMP, (nucleotides) & IMP (inosine monophosphate)
- HGPRT’ase Defeciency
-
Decrease in the nucleotide pool
results in decreased feedback inhibition
of PRPP Glutamine Amidotransferase;
thus, increased nucleotide synthesis
and increased degradation which
elavates uric acid levels
causing uric acid crystal formation. - What disease is associated with Adenisine deaminase
- SCID (severe combined immunodeficiency
- What diseases is associated with HGPRT
-
-Lesch-Nyhan Syndrome
-gout
- hypouricemia - What percent of nucleotides go through salvage pathway
- 90%
- Lesch-Nyhan Syndrome
-
-CP
-self mutilization
-hyperuricemia
-early death - HGPRT (HPRT) is associated with what pathway
- salvage pathway
- HGPRT deficiency
-
X linked recessive
shows up in males
young boys (lesch-nyhan) and old men (gouty arthritis) - How do you treat HGPRT defeciency
- inhibitor of Xanthine Oxidase (because Xanthine more soluable than uric acid)
- drug used to inhibit xanthine oxidase
- allopurinol
- 6 mercaptopurine
- anti-neoplastic