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blood physiology
iron metabolism, heme synth./breakdown
16
Physiology
Graduate
09/27/2012

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Term
serum iron
Definition
the concentration of iron found in the blood either free or bound to transferrin
Term
sideroblastic anemia vs.
sideroblast
vs. hemosiderin
vs. hemosiderosis
Definition
sider refers to iron
hemosiderin stores iron
hemosiderosis is iron overdose leading to hemosiderin deposition
sideroblast is a cell without good heme synthesis - so iron accumulates in mitochondria/ferritin granules.
Term
what are the three types of granules stored by platelets?
Definition
a) coagulation and adhesion factors
d) (dense bodies) - ADP, serotonin, Ca - to vasoconstrict
y) lysosomes and hydrolytic enzymes - clot breakdown
Term
name for RBCs with irregular shapes
- ""irregular sizes
Definition
poikilocytosis
-anisocytosis
Term
which clotting factors are nonenzymatic?
Definition
i) Tissue Factor, ii) Fibrinogen/Fibrin, iii) Factors VIIIa, Va, XIIIa.
Term
thrombomodulin
1) site of production
2) effects
Definition
1) endothelial cells
2) cofactor for thrombin to activate protein C which cleaves factors VIIIa, Va
Term
catylizes ALA -> pyrrole
Definition
porphobilinogen synthase (dehydratase)
Term
which steps of heme synthesis are in the mitochondria?
Definition
ala synthase and ferrocheletase (and the oxidation steps just prior to the ferrocheletase)
Term
enzyme deficiencies lead to pain on light exposure
Definition
porphyrinogen synthases/oxidases/decarboxylases
Term
lead poisons these steps of porphyrin synthesis
Definition
ala synthase, porphyrinobiligin synthase, ferrocheletase
Term
heme oxygenase
Definition
converts heme to biliverdin using O2, and releases CO and Fe 3+
Term
biliverdin reductase
Definition
uses NADPH to reduce biliverdin to -rubin. (reduces central alkene)
Term
solubilization of bilirubin?
Definition
-transferred to liver in albumin complex
- 2 UDP-glucoses are dehydrogenated to UDP-glucuronates
- these are attached to propionate groups in the middle of the bilirubin
Term
which heme breakdown product is secreted in stool?
-in urine (excess breakdown products)
Definition
stercobilin
-stercobilinogen
Term
in which organs does heme oxygenase reaction take place?
Definition
spleen, liver, bone marrow
Term
catalyzes four pyrroles -> tetrapyrrole
Definition
hydroxymethylbilane synthase
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