What is hemoglobin composed of?
4 heme, 4 iron, 4 globins
What disorder does a defect in heme synthesis lead
... [Show More] to?
Porphyria
Porphyria
Deficiency in the enzymes of the porphyrin pathway leads to insufficient production of heme. The principal problem in these deficiencies is the accumulation of porphyrins, the heme precursors, which are toxic to tissue in high concentrations.
What is methemoglobin?
Methemoglobin is a form of hemoglobin that contains ferric [Fe3+] iron and has a decreased ability to bind oxygen. However, the ferric iron has an increased affinity for bound oxygen. When methemoglobin concentration is elevated in red blood cells, tissue hypoxia can occur.
What is the function of the enzyme methemoglobin-cytochrome C reductase in a red blood cell?
To keep iron in the ferrous state
What product is formed when ferrous iron of hemoglobin is oxidized to the ferric state?
Methemoglobin
What is carboxyhemoglobin?
A stable complex of carbon monoxide and hemoglobin that forms in RBCs when carbon monoxide is inhaled or produced in normal metabolism. Large quantities of it hinder delivery of O2 to the body. Tobacco smoking (through carbon monoxide inhalation) raises the blood levels of COHb by a factor of several times from its normal concentrations.
Which hemoglobin has a 200 times greater binding capacity for the hemoglobin molecule than oxygen?
Carboxyhemoglobin
Which hemoglobin derivative causes irreversible changes in RBCs?
Sulfhemoglobin -- makes blood incapable of carrying oxygen.
Which of the following hemoglobins is NOT normally present in a normal adult?
Hb-S
Which embryonic hemoglobin is normally found in newborns?
Hb-F
What is the most abundant hemoglobin in a newborn?
Hb-F
What is the molecular structure of Hemoglobin S?
Valine replaces glutamic acid in the 6th position of the beta chain
Which of the following hemoglobins is insoluble under lowered oxygen tension?
Hb-S
What laboratory test is used to confirm the specific diagnosis of sickle cell anemia?
Hemoglobin electrophoresis
In the solubility test for hemoglobin S, what is the reagent responsible for the reduction of the hemoglobin molecule?
Sodium dithionite
In hemoglobin C, what replaces glutamic acid?
Lysine
Which red blood cell inclusion is characteristic of hemoglobin C disease?
Rod-shaped crystals
What is hemoglobin F composed of?
2 alpha and 2 gamma chains
Which hemoglobin is resistant to alkali denaturation?
Hb-F
Which hemoglobins are differentiated by the Kleihauer-Betke technique?
Hb-A1 from Hb-F
What is the order of migration of hemoglobins, from fastest to slowest, on cellulose acetate at pH 8.4?
A1, F, S, C [Show Less]