摘要:

Hemoglobin Kenitra is a new variant of theβchainα2β269 (E13) Gly→Arg which does not produce any clinical symptoms. It is a slow-moving hemoglobin with a distinctive pattern of electrophoretic mobilities. The stability test was negative. Oxygen affinity studies were not performed. It was found in association withα-thalassemia and microcytosis, but paradoxically a high expression of the variant (55%) was observed.

ISSN:0363-0269    

DOI:10.3109/03630268508996977

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

摘要:

An unstable hemoglobin was detected by isopropanol and heat precipitation tests in a 49-year-old Japanese man suffering from acute exacerbation of a chronic hemolytic disorder which was apparently triggered by infection of cholelithiasis. One of his two sons carried the same abnormal hemoglobin, and was jaundiced, but otherwise healthy, without anemia. The abnormal hemoglobin focused at a slightly more anodic position than Hb A in thin layer polyacrylamide gel electrofocusing. The abnormalβchain emerged after normalβchain in reverse phase high performance liquid chromatography of the hemolysate. It comprised 16.7 % and 25.5 % of the totalβchain in the propositus and his son, respectively.The partially heme-depleted abnormalβsubunit was precipitated with p-chloromercuribenzoic acid, and the abnormalβchain was isolated by urea CM-cellulose column chromatography. Structural analysis demonstrated substitution of proline for histidine at position 97 (FG4) in the e chain.The abnormal hemoglobin was purified by ion-exchange column chromatography. It showed a hyperbolic oxygen equilibrium curve indicating a high oxygen affinity and the absence of cooperative intersubunit interaction. Subunit dissociation seemed to be slightly enhanced. The variant was markedly susceptible to oxidation and rapidly lost heme upon oxidation.

ISSN:0363-0269    

DOI:10.3109/03630268508996978

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

摘要:

Hb F-Tokyo with a Valine→Isoleucine replacement at positionγ34(B16) is a Gγchain variant which was discovered by reverse phase chromatography as this method permitted the nearly complete separation of the three types ofγchain. The chemical characterization was greatly facilitated by the use of a larger, preparative, HPLC column which allowed the isolation of sufficient quantities of the differentγchains.

ISSN:0363-0269    

DOI:10.3109/03630268508996979

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

摘要:

A hemoglobin variant was identified as hemoglobin Mobile in which valine replaces the normal aspartic acid atβ73. studies of its oxygen equilibria and of its interactions in gelation when mixed with hemoglobin S were carried out. Hemoglobin Mobile had an oxygen affinity lower than that of hemoglobin A, as observed by others. However, in mixtures with hemoglobin S, hemoglobin Mobile appeared to impair gelation or increase solubility to a slightly greater extent than did hemoglobin A. Beta73 is a known site of intermolecular interactions in polymers of hemoglobin S. Our studies suggest that the impairment of hemoglobin S polymer formation by altered intermolecular interactions is significantly less in Hb Mobile than in Hb Korle-Bu in whichβ73 is asparagine.

ISSN:0363-0269    

DOI:10.3109/03630268508996980

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

摘要:

The unusual combination of haemoglobins S and E was found in two Saudi Arab siblings. The father was homozygous for HbE and the mother was heterozygous for HbA and HbS. Clinical and laboratory findings are presented and compared with those of the six cases of HbSE heterozygosity previously reported. The significance of the HbE gene as a demographic marker in the Middle East is discussed.

ISSN:0363-0269    

DOI:10.3109/03630268508996981

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

摘要:

A high performance cation exchange system is described for isolating globin chains. The method is rapid and inexpensive, using total lysate, a 14 min gradient, and a 3 cm column. The running buffers contain 6 M urea to dissociate and unfold the globin, and a NaCl gradient is employed for elution. The procedure provides a convenient way of detecting mutant globins with an intrinsic alteration in their charge.

ISSN:0363-0269    

DOI:10.3109/03630268508996982

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268508996983

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268508996984

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268508996985

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268508996986

出版商:Taylor&Francis    

年代:1985

数据来源: Taylor