摘要:

The structure, properties and function of, and some bio-synthetic and genetic aspects of, Hb A2are described. The structural variants of the A2are reviewed and their geographical distribution presented. Hb A2', Hb A2-Flatbush and Hb A2-Babinga are characteristic of negro populations and may have originated in Western or Central Africa. Hb A2-Sphakia is characteristic of Canadian Amerindian and lib A2-Indonesia of Indonesian/Malay populations. Hb A2-NYU has only been found sporadically and most frequently in persons of Eastern European origin. The other three variants of Hb A2have only been reported in a single person or in single families. Some conditions which are associated with changes in Hb A2levels are reviewed.

ISSN:0363-0269    

DOI:10.3109/03630267708999171

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

A 22 year old American negro with mil.d anemia was found to be triply heterozygous for henoglobin S. hemoglobin 0 Arab and alpha thalasseaia. Hemoglobin A was not detected in the subject's hemolysate. The alpha thalasseaia gene was expressed by anα/non-αsynthesis ratio of 0.71à0.07 and was equally unbalanced in the peripheral blood and the bone marrow. The total radioactivity of the abnormal chains was equal, indicating equal rates of transcription and translation of the abnormal structural genes. Since the rate of synthesis of BSand B0was equal to the proportions of these abnormal chains in the peripheral blood, the abnormal gene products were equally stable. The mildness of the anemia, compared to that of the other reported S-O Arab double heterozygotes, may be due to the moderating influence of the alpha thal gene.

ISSN:0363-0269    

DOI:10.3109/03630267708999172

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

A 25 year old woman with congenital dyserythropoietic anemia (CDA) Type I is described. Typical morphologic abnormalities of the erytliroid precursors in the bone marrow by light and electron microscopy, marked ineffective erythropolesis and iron loading were present. Globin chain synthetic ratios as well as functional and structural studies on the patient's hemoglobin were normal, ruling out the presence of thalassemia or a mutant hemoglobin which can both give rise to morphologic and clinical features similar to CDA. The laboratory findings on this patient and family members and a brief review of the literature are presented.

ISSN:0363-0269    

DOI:10.3109/03630267708999173

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

Virtually all protoporphyrin in erythrocytes of patients vith erythropoietic protoporphyria is bound to hemoglobin. The maximum of the fluorescence excitation spectrum of this protoporpliy-rin-hemoglobin complex shifted, with increasing concentration, from 405 nm to 389 nm. A similar shirt was observed wlien titrating a solution of free protoporphyrin with hemoglobin. The Soret maximum of free protoporphyrin itself. on the other hand, was not concentration-dependent. These observations indicate that spectrofluorometric measurements do not allow conclusions coticerning the mode of protoporphyrin binding to hemoglobin.Experiments on protoporphyrin exchange between the hemoglobins A, F and S reinforced the previously drawn conclusion that protoporphyrin is bound to hemoglobin at the heme-binding sites.

ISSN:0363-0269    

DOI:10.3109/03630267708999174

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267708999175

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267708999176

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267708999177

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267708999178

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267708999170

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor