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560D COMP EXAM PREP LATEST UPDATED
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560D COMP EXAM PREP LATEST UPDATEDG6PD deficiency
Low NADPH leads to hemolytic anemia (free radicals) with bite cells and heinz
bodies
Fava beans, sulfonamides, primiquine, dapsone
X-linked recessive
Sideroblastic anemia
anemia is caused by inability to use available iron to manufacture hemoglobin
iron deficiency anaemia
anemia resulting when there is not enough iron to build haemoglobin for red
blood cells
B12 anemia
Pernicious anemia- results when people cannot make intrinsic factor
*megalobast RBC
*leads to death
folate deficiency anemia
lack of folate leading to premature RBC death... caused by dietary deficiency
Iron as part of heme
heme/porphyrin pathway
fenton reaction
Allows iron to generate hyroxyl free radicals
Fe2+ + H2O2 --> Fe3+ + OH- + OH.
Bohr effect
, a decrease in the amount of oxygen associated with hemoglobin and other
respiratory compounds in response to a lowered blood pH resulting from an
increased concentration of carbon dioxide in the blood.
DMT1 (divalent metal transporter 1)
transports ferrous iron into the cell
*also transports zinc, copper, manganese so any excess can inhibit absorption
of other metals
folate trap
No B12 so Methyl-THF isn't converted to regular THF so buildup of methyl-THF
and homocysteine (as homocysteine is converted to methionine) -
megaloblastic anemia
delta-aminolevulinic acid synthase
key regulator for heme synthesis
Porphyrinogen vs Porphyrin
porphyrin: any of a class of heterocyclic compounds containing four pyrrole
rings arranged in a square; they are important in biochemistry in a form with a
metal atom in the central cavity (hemoglobin with iron, chlorophyll with
magnesium, etc)
porphyrinogen: a reduced form of porphyrin that is an intermediate in the
biosynthesis of heme
Porphyrin and Heme Synthesis pathway
how does a lack of carbs affect patients with porphyrias
Degradation of heme to bilirubin
-excretion products
ALT
AST
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