摘要:

Polyacrylamide gel electrophoresis (PAGE) in the presence of urea, acid, and Triton X-100 was used for determination of theGγtoAγratio in human Hb F. The data compared most favourably with results obtained by a HPLC procedure and by a chemical procedure. Moreover, its accuracy and reproducibility was determined. The presence of Hb A2in a sample with Hb F level below 10% interferes with the determination because of the nearly identical electro-phoretic mobilities of theδandGγchains. Thus, the removal of Hb A2is required. Samples free of Hb A2but with a Hb F level as low as 2% can be analyzed with an accuracy of about 5%. The PAGE method has been applied to the Hb F of 63 subjects withβthalassemia and related conditions, and the results of theseanalysesare included in this communication.

ISSN:0363-0269    

DOI:10.3109/03630268108991832

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

A new unstable hanoglobin was discovered in a Japanese boy without any signs of hemolytic disease during screening of patients by the isopropanol test. The abnormal hemoglobin was found in three generations. This slightly unstable hemoglobin could not be separated from Hb A by electrophoresis. All carriers showed an increased whole blood oxygen affinity. Structural studies demonstrated a previously unreported amino acid substitution,β23[B5] valine→glycine. This new unstable variant has been named Hb Miyashiro.

ISSN:0363-0269    

DOI:10.3109/03630268108991833

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

A new unstable hemoglobin was found in a 33-year-old Japanese woman with a history of chronic, partially compensated hemolytic anemia. An electrophoretically silent unstableβchain was prepared by PCMB precipitation and the amino acid substitution determined asβ31(B13)Leu→Pro. Her son was a carrier of the same variant, who manifested milder symptoms.

ISSN:0363-0269    

DOI:10.3109/03630268108991834

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

A fast-moving hemoglobin variant was found in five members of a Chinese family of the Wuming district. The relative amount of thisαchain variant in the heterozygote was about 20%. The abnormality caused no ill effects in its carriers. Sequence analysis identified a Lys→Gln substitution at positionα-11 (A9).

ISSN:0363-0269    

DOI:10.3109/03630268108991835

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268108991836

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

An unstable hemoglobin variant was found in a 24-year-old Chinese of Han origin. It had an electrophoretic mobility slightly faster than that of Hb A2, but could not be separated from it completely by cellulose acetate elec-trophoresis (pH 8.6). Chromatography on a DE-52 column showed that the variant amounted to approximately 13% of the total hemoglobin.Structural analysis showed that the hemoglobin had the structureα27(B8) Glu→Lys. It was named Hemoglobin Shuangfeng. Theα27 Glu residue is an invariable residue located near theα1βlcontact.

ISSN:0363-0269    

DOI:10.3109/03630268108991837

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

Hemolysate from adult and newborn rats was fractionated into nine components (I-IX) using DEAE-cellulose chromatogra-phy. A subsequent chromatographic step resolved component IV into two, IVa and IVb. A comparison of adult and newborn rat hemolysates showed that newborn had higher levels of components I, 11, and IV. In the newborn, they accounted for 52% of total hemoglobin eluted, compared to 34% in the adult. Components V, VI, and VIII were less in the newborn than in the adult red cell, at 42% compared to 59%. Minor components 111, VII, and IX were unchanged, at 5.8% in newborn and 5.3% in adult rats. Bone marrow cells from adult rats cultured for 46 hours, showed stimulation of eight components under the influence of erythropoietin (components IVa and IVb were not distinguished, and VII was not detected). The extent of stimulation was not consistently higher for major than for minor hemoglobins. These data show that adult rats possess at least ten hemoglobin components, that newborn rats have the same components but in different proportions, and that both major and minor components are stimulated by erythropoietin in bone marrow cell cultures.

ISSN:0363-0269    

DOI:10.3109/03630268108991838

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

摘要:

Hemoglobin Cubujuqui (1) was detected in several members of a Mexican-American family. Structural analysis of this hemoglobin indicated that the carboxyl terminal arginine at position 141 in theαchain had been replaced by serine. This residue is critical not only in stabilizing the deoxy or T conformation by electrostatic interactions, but it is also involved in the Bohr effect through its linkage with Val lαof the opposite a chain in the tetramer. The variant exhibits high affinity for oxygen that is associated with destabilization of the deoxy conformation, and reduced cooperativlty. The pH and the chloride sensitivity of the variant are also reduced, as compared to Hb A.

ISSN:0363-0269    

DOI:10.3109/03630268108991839

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268108991840

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630268108991841

出版商:Taylor&Francis    

年代:1981

数据来源: Taylor