Pyrimidine Synthesis Flashcards
Where does ribose phosphate come from?
HMP shunt
What is the rate limiting step of pyrimidine synthesis?
Glutamine into Carbamoyl Phosphate (enzyme: carbamoyl phosphate synthetase II)
- ATP ->ADP
- Co2
What enzyme catalyses the conversion of Glutamine to Carbamoyl Phosphate?
Carbamoyl phosphate synthetase II
Where is carbamoyl phosphate I found (what is it involved in)?
Found in mitochondria
- Urea cycle
What is Carbamoyl phosphate synthetase II inhibited by?
UMP
What is formed first the ring or the ribose sugar?
Ring formed first then ribose sugar
- Purines is the opposite
What is carbamoyl phosphate converted into?
Orotic acid (
What 2 molecules are combined to make UMP?
- Orotic acid (ring/base)
- PRPP (Sugar + Phosphate)
Enzyme: UMP synthase
What is the bifunctional (catalyses 2 steps) enzyme that convertes Orotic acid and PRPP to UMP?
UMP synthase
What molecules make up the pyrimidine ring?
- Carbamoyl phosphate (1N, 1C)
- Aspartate (1N + 3C)
What enzyme is defective in orotic aciduria?
UMP synthase
How is orotic aciduria inherited?
Aut Recessive
What nucleotide is made first (what are the 2 derived from)?
UMP
What are the key findings/features in orotic aciduria
- Orotic acid in urine
- Loss of pyrimidines
- Megaloblastic anemia
- No B12/folate response
- Growth retardation
What is orotic aciduria treated with?
- Uridine
As it bypasses UMP synthase