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48 Cards in this Set

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  • Back
What is the enzyme in the committed step for purine synthesis?
Glutamine phosphoribosyl amidotransferase
How does caffeine work?
It is a competative inhibitor of adenosine, which has inhibitory effects on the CNS
Most de novo synthesis of purines occurs in what ogan?
Liver
How many high energy bonds does purine synthesis require?
6
How many enzymes use ATP in purine sythesis?
4
What is the first purine nucleotide in the synthesis of purines?
Inosine Monophosphate (IMP)
The base of IMP is what?
hypoxanthine
What is the key enzyme in the purine salvage pathway?
hypoxanthine-guanine phosphoribosyltransferase
Name a cell type where the salvage of bases is the major form of nucleotide generation.
lymphocytes
Deficiency in purine nucleoside phosphorylase (PNP) can lead to what?
Immunodeficiency disease, specifically T cell immunity is compromised
Lesch-Nyhan Syndrome is caused by deficiency in what enzyme?
HGPRT for example inhibition of Guanine to GMP
What can treat Lesch-Nyhan Syndrome?
Allopurinol
What happens with adenosin deaminase deficiency?
Severe combined immunodeficiency disease (SCID)
Deficiency of AMP deaminase in muscle results in what?
Muscle fatigue during exercises
What muscle specific enzyme can create ATP directly from ADP?
myokinase
What is CAD in reference to the synthesis of pyrimidines?
First three enzymes are located on the same polypeptide (carbamoyl phsophate synthetase II, Asp transcarbamoylase, and Dihydro-orotase
Deficiency of the second enzyme (ornithine transcarbamoylase) of the urea cycle can lead to what by passing CPSII?
orotic acidemia
Deficiency of UMP synthase, which takes ortic acid to UMP, does what?
Blocks pyrimidine synthesis, DNA replication doesn't work and leads to growth retardation; also cause ortic aciduria
How do you treat UMP syntase deficiency?
treat with oral administration of uridine: uridine converted to UMP bypassing the metabolic block
What anticancer drug inhibits dihydrofolate in DNA synthesis
Methotrexate and 5-fluorouracil (5-FU)
Do we rely on the slavage pathway of pyrimidines much?
No
What enzymes is used to reduce ribose to deoxyribose?
ribonucleotide reductase
What activates ribonucleotide reducatase and inactivates it?
ATP activates the enzyme and dATP inhibits
High levels of dTTP we get…?
Promtoes conversion of GDP to dGDP and promotes ADP to dADP and then get elevated dATP, which inhibits the enzyme
ATP inhibits or stimulates production of deoxyribonucleotides?
Stimulates and dATP inhibits
Degradation of purines occure in what organ?
liver
Xanthine is converted to uric acid with what enzyme
Xanthine oxidase
xanthine oxidase also converts what before uric acid?
it converts hypoxanthine to xanthine before uric acid
Guanine to Xanthine uses what enzyme?
Guanase
What two enzymes make xanthine?
Guanase and xanthine oxidase
Urate is also used as an antioxidant?
yes
Two causes of accumulation of uric acid
overproduction or malsecretion
What in inhibits of xanthine oxidase?
Allopurinol
Why limit use of alcohol with gout?
Alcohol increases the use of ATP and leads to high degradatin of purines
Hereditary orotic aciduria is caused by what?
UMP syntase and you accumulate orotic acid and get growth retardation
What is UDP-glucuronic acid involved in?
Bilirubin conjugation, synthesis of polysaccharides and oligosaccharide chains of glycoproteins
What coenzymes Is Adenosine a core element of?
NAD, FAD, CoA, ATP, GTP, etc.
What inhibits CPS II?
UTP
What drug inhibits thymidylate synthase?
5-fluorouracil
What drug inhibits dihydrofolate reductase?
Methotrexate
What are the byproducts of pyrimidine degradation?
NH4+, beta-alanine, CO2, B-aminoisobutyrate
What converts bases to their nucleosides in pyrimidine salvage?
nucleoside phosphorylase
What conversts nucleosides to nucleotides in pyrimidine salvage?
specific nucleoside kinases
Thymine phosphorylase does what?
It adds a deoxyribose resideu to make Thymidine (nucleoside) in pyrimidine salvage
Reduction of ribose to deoxyribose occurs at what level of nucluotides?
At the diphosphate level
Ribonucleotide reductase has how many allosteric sites?
Two
Ribonucleotide reductase requires what addition enzyme with what cofactor?
thioredoxin reductase and NADPH
What is an attack treatment of gout?
NSAIDs, such as ibuprofen or injection of glucocorticoids into joints