Biochem Flashcards
All amino acids undergo transamination except
Lysine
Hydroxyproline
Proline
Threonine
Coenzyme for transaminases
B6
Coenzyme for transketolases
2nd phase of PPP
B1
Substrates of urea cycle
CO2
Aspartate
NH3
Rate-limiting step of urea cycle
CPS 1
Reactions occuring both in the mitochondria and cytosol
Heme synthesis
Urea cycle
Gluconeogenesis
How many moles of ATP are needed in the synthesis of urea?
3 moles of ATP
4 high-energy bonds
Most common enzyme defect in urea cycle
Ornithine transcarbomylase deficiency
Most severe enzymatic defect in urea cycle
CPS I deficiency
phenylketonuria results from deficiency of what enzyme/coenzyme
phenylalanine hydroxylase
tetrahydrobiopterin
coenzyme for branched chain AA dehydrogenase
B1
coenzyme for pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase
B1
enzymes of urea cycle
mitochondria:
CPS I
ornithine transcarbomylase
cytoplasm:
arginosuccinate synthetase
arginosuccinate lyase
arginase
where does HMP shunt occurs
in the cytosol
RBCs and tissues that produce lipids (liver, adipose, adrenals, thyroid, testes, lactating mammaries)
key enzyme of the 1st phase of HMP shunt
G6PD
which phase of the HMP shunt yields 2 NADPH
Phase 1
which phase of the HMP shunt is oxidative and irreversible
first phase
condition characterized by kinky hair and growth retardation due to deficiency of copper required by lysyl oxidase
Menkes disease