HMP Flashcards
Oxygen free radicals (reactive oxygen species) are produced using what enzyme
NADPH oxidase
Enzyme that catalyzes H2O2 formation from ROS?
superoxide dismutase
Deficiency of NADPH oxidase results in what disease
chronic granulomatous disease
Vitamin required in nonoxidative/reversible HMP shunt
B1
Products of oxidative reaction?
CO2
(2) NADPH
Ribulose-5-P
Enzyme in nonoxidative reaction?
phosphopentose isomerase
transketolases
Heinz bodies seen in what disease
G6PD deficiency
Heinz bodies are formed by what
HEinz = denatured HEmoglobin
Formation of bite cells:
splenic macrophages phagocytose Heinz bodies of RBCs, forming bite cells
CGD patients are susceptible to infection by what?
catalase-positive organisms
In chronic granulomatous disease patients, hydrogen peroxide cannot be produced by macrophages (due to NADPH oxidase deficiency). H2O2 is taken from the enviroment. Catalase neutralizes H2O2 and converts it to water.
Enzyme catalyzing this reaction H2O2 + GSH –> H2O + GSSG
hydrogen peroxide reacts with reduced glutathione to form water and glutathione disulfide.
catalyzed by GLUTATHIONE PEROXIDASE
Deficiency in fructokinase:
essential fructosuria
Deficiency in aldolase B:
fructose intolerance
decreased aldolase B
increased F-1-P
decreased available PO4
inhibited gluconeogenesis, glycogenolysis
Galactose enters glycolysis by:
UridylTransferase (G1P uridyltransferase)
GUT
Fructose enters glycolysis by:
Aldolase B
FAB