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18 Cards in this Set

  • Front
  • Back
What is the HMP shunt used for?
Provides source of NADPH from abundantly available G6P
Where does the HMP shunt occur?
Lactating mamillary glands, liver, adrenal cortex, RBC's
What is the respiratory burst used for?
Immune response --> rapid release of reactive oxygen intermediates
How do WBC's protect themselves from the components of the respiratory burst?
Converts hydrogen peroxide to water
What is necessary for glutathione to detoxotify free radicals and peroxides?
NADPH
What happens to RBC's with decreased NADPH?
Leads to hemolytic anemia due to poor RBC defense against oxidizing agents
What are heinz bodies?
Oxidized hemoglobin precipitated within RBC's
What are Bite cells?
Result from phagocytic removal of heinz bodies by splenic macrophages
What is the pathology of essential fructosuria?
Involves a defect in fructokinase
What are the symptoms of essential fructosuria?
Fructose in blood an d urine
What is the pathology of fructose intolerance?
Hereditary deficiency of Aldolase B
What are the symptoms of fructose intolerance?
Fructose-1-P accumulates, causing a decrease in available phosphate
How do you treat fructose intolerance?
Dec intake of both fructose and sucrose
What is the pathology of galactokinase deficiency?
Hereditary deficiency of galactokinase
What are the symptoms of galactokinase deficiency?
Galactose appears in blood and urine, infantile cataracts
What is the pathology of galactosemia?
Absence of galactose-1-phosphate uridyltransferase
What are the symptoms of galactosemia?
Damage cause by accumulation of toxic substances which accumulate in the lens of the eye
Failure to thrive, jaundice, hepatomegaly, infantile cataracts, mental retardation
How do you treat galactosemia?
Exclude galactose and lactose from diet