Two human hemoglobins designed to inhibit the polymerization of sickle hemoglobin (Hb S; alpha 2 beta S2) have been produced. Mutations that disrupt the ability of Hb S to form polymers were introduced into the normal human beta-globin gene by site-specific mutagenesis. These mutations affect the axial and lateral contacts in the sickle fiber. The recombinant hemoglobin designated anti-sickling...

The existence of a Kodaira fibration, i.e., of a fibration of a compact complex surface S onto a complex curve B which is a differentiable but not a holomorphic bundle, forces the geographical slope ν(S) = c 1 (S)/c2(S) to lie in the interval (2, 3). But up to now all the known examples had slope ν(S) ≤ 2 + 1/3. In this paper we consider a special class of surfaces admitting two such Kodaira fi...

Background: The frequency of pyruvate kinase (PK) deficiency, an autosomal recessive defect, is approximately 3 per 10,000 individuals in Shiraz and surrounding areas, and is increased due to high consanguinity marriage frequency. The purpose of this study is to obtain data on the frequency and spectrum of gene mutation of PK in newborns, from Shiraz and surrounding areas. Materials and Methods...

The recessive mouse mutant headbobber (hb) displays the characteristic behavioural traits associated with vestibular defects including headbobbing, circling and deafness. This mutation was caused by the insertion of a transgene into distal chromosome 7 affecting expression of native genes. We show that the inner ear of hb/hb mutants lacks semicircular canals and cristae, and the saccule and utr...

OBJECTIVE To evaluate the results and cost-effectiveness of prenatal prevention measurement in severe thalassemia diseases at Srinagarind Hospital. STUDY DESIGN Descriptive study. SETTING Antenatal care (ANC) Clinic, Srinagarind Hospital, Faculty of Medicine, Khon Kaen University. SUBJECTS 1,498 thalassemic screened pregnant women first presenting at ANC Clinic at gestational age less tha...

Hemolysis can saturate the hemoglobin (Hb)/heme scavenging system, resulting in increased circulating cell-free Hb (CF-Hb) in hereditary and acquired hemolytic disease. While recent studies have suggested a central role for intravascular hemolysis and CF-Hb in the development of vascular dysfunction, this concept has stimulated considerable debate. This highlights the importance of determining ...

BACKGROUND Elevation of haemoglobin (Hb) with recombinant erythropoietin (rHuEpo) in patients with chronic renal failure has raised concern of increased risk of thromboembolic diseases. In this study, a substudy of the Scandinavian multicentre trial, we examined the influence on haemostatic parameters of normalization of Hb levels from subnormal levels in patients with chronic renal failure. ...

Hemoglobin S in homozygous state or in combination with one of the structural variants of Hb D-Punjab, Hb O-Arab, Hb C or β-thalassemia mutation results in sickle cell disease (SCD) that is characterized by chronic hemolytic anemia and tissue injury secondary to vasooclusion. A chronic hypercoagulable state in SCD has been established with the increased risk of thromboembolic complications in t...

The present study was conducted in the Outpatient Department (OPD) of Yangon Children's Hospital (YCH) during June to November 1990 to determine the hematological data of 133 Myanmar patients with thalassemia trait who were the parents of patients with known beta-thalassemia major or hemoglobin E (Hb E)/beta-thalassemia. The mean values of hemoglobin (Hb) concentration, packed cell volume (PCV)...

Computerized molecular model building has been used to deduce the arrangement of sickle cell hemoglobin molecules (Hb-S) in the tubular fibers which form within sickling cells and in concentrated cell-free solutions of deoxygenated Hb-S. A "best" solution has been found which satisfies all of the reported properties of these fibers. In the proposed arrangement the contact between adjacent Hb-S ...