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23 Cards in this Set
- Front
- Back
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how is sickle cell disease inherited
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autosomal recessive
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what substitution occurs in HbS in the protein structure
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glutamyl residue at position 6 of HbA beta chain replaced by valine
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in the DNA, what change occurs to result in HbS
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A to T
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prenatal diagnosing can be done by isolating material from...
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amniotic cells or trophoblastic cells (chorionic villi sampling)
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what restriction endonuclease is used to test for HbS
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Mst II
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if the sickle cell trait is present, the resulting DNA fragment will be...
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longer
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PCR product of hemoglobin digests can be analyzed by ___ hybridization
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allele-specific oligonucleotide
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the presence of valine at position 6 of HbS dramatically reduces the solubility of ___ HbS
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deoxy
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what happens to HbS in the deoxy state
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polymerizes
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deoxy HbS polymeriztion is inhibited by...
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HbF
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in sickle cell disease, cloride is transfered with ___ out of the cell
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potassium
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SS red cells contain cellular ___ molecules not found on normal red cells
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adhesion
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what three examples of supportive care can be given to individuals with HbS
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prophylactic penicillin, transfusions, NO
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should tranfusions be done in cases of usual anemia or episodes of pain
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no
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what can be done to the solubility of HbS to help patients with this disease
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increase solubility
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what can be given to affect the RBC membrane to induce swelling
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cetiedil and monensin
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what drug will inhibit the Gardos Potassium channel to increase RBC swelling
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clotrimazole
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what compound will inhibit the potassium-chloride channel to increase RBC swellin
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Mg
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what drug can increase HbF in sickle cell patients
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hyroxyurea
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what type of transplant can be done to nearly cure (80%) all of HbS
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bone marrow
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which two factors affect HbS polymerization the most
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oxygen concentration (low) and HbS concentration (high)
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the riate of polymerization of HbS is proportional to the ___ power of the effective deoxy HbS concentration
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15
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gamma globin has a ___ residue at position 87 (F3) instead of ____ in the beta globin
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glutamine/threonine
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