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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
24
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pubmed:dateCreated |
1987-2-6
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pubmed:abstractText |
We have found a sickling variant, Hb S Antilles, alpha 2 beta 2(6 Glu----Val, 23 Val----Ile), that has the same electrophoretic mobility as Hb S but a distinct isoelectric focus and produces sickling in the carriers of the Hb A/S Antilles trait. The carriers' erythrocytes tend to sickle at O2 partial pressures similar to those that induce sickling in Hb S/C disease. Pure deoxy-Hb S Antilles is 50% as soluble as deoxy-Hb S (saturating concentration = 11 g X dl-1 compared to 18.4 for Hb S). Dilute solutions of pure Hb S Antilles have a lower oxygen affinity than those of Hb A or Hb S (partial pressure for 50% binding is 9 mm Hg compared to 5.5 mm Hg for Hb A or S at pH 7.00). A/S Antilles erythrocytes have a much lower oxygen affinity than A/S cells; this is further decreased in dense cells fractionated on a Percoll density gradient. Their oxygen equilibrium curves had anomalous shapes like those of S/S cells. Fiber formation in the erythrocytes of Hb S Antilles carriers is clearly due to its low solubility and oxygen affinity, showing that heterozygosity for this hemoglobin presents another sickle cell syndrome and suggesting that Hb S heterozygotes who exhibit symptoms of sickle cell disease should be carefully screened for double mutations.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-1052169,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-1225575,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-14233155,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-19257,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-22759,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-27380,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-3701868,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-3841063,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-3965046,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-468817,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-478976,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-5967288,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-6016610,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-6310398,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-638255,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-6493211,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-7034266,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-7105414,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-7161106,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-7338468,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-7437513,
http://linkedlifedata.com/resource/pubmed/commentcorrection/3467311-927175
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Dec
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pubmed:issn |
0027-8424
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
83
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
9363-7
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:3467311-Amino Acid Sequence,
pubmed-meshheading:3467311-Anemia, Sickle Cell,
pubmed-meshheading:3467311-Erythrocytes, Abnormal,
pubmed-meshheading:3467311-Gels,
pubmed-meshheading:3467311-Globins,
pubmed-meshheading:3467311-Hemoglobin, Sickle,
pubmed-meshheading:3467311-Heterozygote,
pubmed-meshheading:3467311-Humans,
pubmed-meshheading:3467311-Hydrogen-Ion Concentration,
pubmed-meshheading:3467311-Isoelectric Point,
pubmed-meshheading:3467311-Mutation,
pubmed-meshheading:3467311-Oxyhemoglobins,
pubmed-meshheading:3467311-Solubility
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pubmed:year |
1986
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pubmed:articleTitle |
Hemoglobin S Antilles: a variant with lower solubility than hemoglobin S and producing sickle cell disease in heterozygotes.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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