Roth Flashcards
which drug used for trx of patient with overproduction of uric acid
allopurinol
inhibits xanthine oxidase
lesh nyhan
- inheritance
- sx
x linked r
hyperuricemia, spastic cerebral palsy, MR, self mutilation
3 AA to synthesize purine ring
glycine, aspartate, glutamine
enzyme deficient in selective T cell immunodef
purine nucleoside phosphorylase
What enzmye is inhibited by…
- 5-FU
- Methotrexate
- Hydroxyurea
TS-thymidilate synthase
-makes dTTP
DHFR-Dihydrofolate reductase
RR-ribonucleotide reductase-makes dXTP
Absnece of which enzyme leads to accumulation of metabolites in purine salvage pathway leading to SCIDS
ADA-adenosine deaminase
absence of this enzyme leads to lesich nyhan
hgprt-hypoxanthine guanine phosphoribosyltransferase
no base salvage of hypoxanthine or guanine
PRPP synthetase inhibitors
purne di/tri nucleotides
committed step of purine syntehsis?
Synthesis of phosphoryibosylamine from PRPP bt PRPP amidotransferase
De novo purine synthesis results in
IMP
@ IMP syntehsis-what can be formed
AMP and GMP
What kind of regulators are AMP and GMP of thier synthesis
negative
trimethoprim mechanism
inhibts bacteria DHFR
Folic acid analog mechanism and result
Inhibits DHFR
-purine and TMP syntehsis
no DNA synthesis-cell death
purine salvage function
take purine bases and turn into nucleoside phosphate residues to recycle
2 enzymes in purine salvage
HGPRT and APRT
ADA def results in
SCID
HGPRT def results in
-Sx?
Lesch Nyhan
-self mutilation, AR, aggresion, hyperuricemia, gout
possible causes of gout
Lesch Nyhan, PRPP excess, decresaed uric acid excretion, G6PDH def, some diuretics
allopurinol results in increased levels of
-what is special about these
hypoxanthine and xanthine
-dont crystalize
pyrimidine synthesis comitted step
carbomyl phosphate fomration
CPS1 vs CPS2
1 depends on N-acetylglutamate as allosteric activator
- invovled in urea cycle
- in mitochondria
- utilize NH3 as nitrogen source
2
- invovled in pyrimidine syn
- cytosol
- utilizes gamma amide group of glutamine as N source
UMP becomes
UTP, CTP
dUMP, TMP
what enzyme makes ribonucleotides to deoxyribonucs
ribonucleotide reductase
what inhibits RR
dATP/hydroxyurea
enzyme def in orotic aciduria
-wgat accumulates
UMP synthase
-contains both ortate phosphoribosyl transferase and OMP decarboxylase activites
orotate accumulates
how trx orotic aciduria
uridine and cystidine
