Gout and Orotic Aciduria
What is Gout?
Which one of the following is not a function of a purine or pyrimidine?
Energy Source
Precursor for Nucleic Acid Biosynthesis
Precursor for cofactor synthesis
Second messengers
Allosteric regulators
Oxygen Transport
Energy Source — ATP is an energy source
Precursor for Nucleic Acid Biosynthesis
Precursor for cofactor synthesis— NAD
Second messengers — cAMP, AMP
Allosteric regulators — AMP
Oxygen Transport
The nucleoside formed when hypoxanthine is the base is:
Zanthine
Hypoxanthosine
Adenosine
Inosine
Inosine
Thymine
Thiamine — comes from dUMP and N5, N10 MethylTHF
Purine Synthesis
2 Different Pathways
Dietary intake of intact Purines is Zero. They are not contributing to our salvage pathway. All dietary Purines are converted to Uric Acid
The formyl carbon in N10 formyl THF cab be derived from which one of the following?
Pyruvate
Glycine
Leucine
Lysine
Glutamate
Glycine — Glycine Cleavage Enzyme — Goes to N5, N10 which can go in two steps to N10 Formyl THF
Tetrahydrofolate
The ribose for purine synthesis can be derived from all of the following EXCEPT for which one?
Glucose
Fructose
Lactate
Succinate
Hydroxybutarate
Hydroxybutarate — KB cannot be used to make sugars. All others are glycogenic.
Synthesis of PRPP
Committed Step of Purine Biosynthesis
Which one of the following could lead to an increase in PRPP Levels?
Type I Diabetes
Von Gierke Disease
McArdle Disease
MCAD Deficiency
G6PDH Deficiency
Type I Diabetes
Von Gierke Disease — G6Phosphatase deficiency— goes through HMP shunt — Gout is a symptom because of elevation G6P
McArdle Disease
MCAD Deficiency
G6PDH Deficiency — G6P doesn’t accumulate as much as G6P.
Outline of Purine Ring Biosythesis
What you should know about Purine DeNovo Pathway
Biosynthesis of AMP from IMP
Biosynthesis of GMP from IMP
Conversion of AMP, GMP to more phosphorylated forms
Regulation of DeNovo Purine Synthesis
Purine Synthesis and Gout
Purine Salvage
Purine Nucleoside Phosphorylase Deficiency
HGPRT Deficiency
Adenosine Deaminase Deficiency
Purine Nucleotide Cycle
What other enzyme reaction aids in buffering?
Pyruvate Dehydrogenase
Glutamine Synthetase
Glutaminase
Pyruvate Carboxylase
Alcohol dehydrogenase
Aldehyde Dehydrogenase
Glutaminase
Serine Derivation and Making THF
Formate degredation can be used to make…
Histidine Degredation can be used to make…
Glycine can make…
dUMP to TMP uses?
Conversion of Glycine to Serine can be used to make…
Homocysteine to Methionine uses…
Purine Biosynthesis requires…