Hemoglobin Gun Hill: an unstable protein associated with chronic hemolysis.
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چکیده
منابع مشابه
Severe hemolytic anemia associated with the homozygous state for an unstable hemoglobin variant (Hb Bushwick).
We have investigated a 13-year-old girl from first cousin parents who presented with severe hemolytic anemia. Hematologic studies showed unstable hemoglobin (Hb) disease (chronic Heinz body anemia), and DNA analysis showed that the patient was homozygous for the previously reported abnormal Hb called Hb Bushwick (beta 74E18 gly-->val). Hb Bushwick is unstable in vitro and in vivo. In addition, ...
متن کاملObservations on the mechanical precipitation of oxy Hb S and other mutants.
Oxyhemoglobin S exhibits greater mechanical instability than oxyhemoglobin A. The rate of precipitation of Hb S when agitated by vortexing depends upon the geometry of the tube, the volume of the hemoglobin solution, and the concentration of hemoglobin. The rate of precipitation is inversely related to concentration. Precipitation is inhibited by temperatures near 4 degrees C and alkylureas who...
متن کامل[Hemolytic anemia due to hemoglobin Evans in an Argentinean family].
Unstable hemoglobins are structural variants of the hemoglobin molecule, mostly originated by single amino-acid replacement in some globin chains. These changes affect molecule stability, leading to loss of solubility, precipitation, and cellular lysis. Patients carrying these unstable hemoglobins may present mild to severe chronic hemolytic anemia. Hemoglobin Evans is an unstable variant origi...
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ABSTRACT Glucose -6-phosphate dehydrogenase (G6PD)deficiency is the most common inheritory enzyme defect in human. With this deficiency, red blood cells are susceptible to hemolysis induced by oxidants .Drage and foods (esp. fava beans) are the most common oxidants that cause acute hemolysis (Favism). Vitamin E.that is an potent antioxidant,was studied in chronic hemolysis of these patients. ...
متن کاملHemoglobin Philly ( p 35 Tyrosine Phenylalanine ) : Studies in
Received for publication 24 January 1969. INTRODUCTION The severity of the clinical manifestations of the different abnormal hemoglobins is varied. Many of these mutant proteins produce no discernible effect on the patient, while some cause a mild compensated hemolysis, and others are associated with severe hemolytic disease (1). The detection of an abnormal hemoglobin in many patients presenti...
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عنوان ژورنال:
- Blood
دوره 32 3 شماره
صفحات -
تاریخ انتشار 1968