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7 Cards in this Set
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What is the amino acid substitution in HB S?
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Beta 6 glumatic acid by VALINE
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What is the amino acid substitution in HB C?
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6 Glutamic acid to lysine
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How does HB F inhibit sickling?
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glumamine residue at gamma 87 prevents critical lateral contact in the double strand of the sickle fibre.
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What ionic abnormalities cause the sickle cells to shrink?
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1. K-Cl transport out of the cell is overactive (due to H+ and swelling) in sickle cells and water is taken out of the cell.
2. sickle cells have more Ca++ than normal (Ca++ influx) |
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what does it mean that sickle cells have a high P50*?
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they must be exposed to a high PO2 to in order to be saturated.
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What is normal P50?
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27 mmHg1.
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What are therapeutic approaches to sickle cell?
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1. lower MCHC (hyponatremia)
2. clotrimazole: inhibit KCl loss (Gardos channel), inhibit co-transport with magnesium salts 3. induce HB F: azacytidine, hydroxyurea, EPO, butyric acid, BM transplant |
