摘要:

The complete primary structure of theβchain from the adult hemoglobin of a baboon,Papio cynocephalus, has been determined by automated, Edman degradation of the intact chain and four fragments derived there from by specific cleavage reactions. The analysis was facilitated by application of a modified solvent system that permits unambiguous identification, by high-performance liquid chromatography, of the 17 amino acids whose phenylthiohydantoin derivatives are soluble in ethyl acetate. The sequence obtained differs from that of the humanβchain at eight sites, a degree of divergence similar to that observed when human and macaqueβchains are compared. Of the cercopithecoidβchains whose complete sequences have been determined or inferred from compositions of small peptides, that ofP. cynocephalusis most like theβchain of the gelada baboon, an observation in accord with assessment of a close phylogenetic relationship between the generaPapioandTheropithecus.

ISSN:0363-0269    

DOI:10.3109/03630268009042379

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

The oxygen binding properties of Hb M Hyde Park (92β, histidine↠tyrosine) were reinvestigated directing special care to testing the wave length-dependence of the oxygen equilibrium curve and to stabilizing hemoglobin samples using a methemoglobin reductase system. There was no indication that the Hb M Hyde Park fraction separated on a DEAE Sephadex column contained an unknown hemoglobin derivative which appeared in earlier studies. Contrary to earlier observations, there was no significant wave length-dependence of the equilibrium curve of Hb M Hyde Park, verifying the spectrophotometry determination of oxygen saturation. The reductase system satisfactorily reduced the normal a chain met hemes without reducing the abnormalβchain met hemes. The oxygen binding property of Hb M Hyde Park is characterized by 3 to 4 times higher oxygen affinity than that for normal hemoglobin, complete loss of cooperativity, and substantially preserved Bohr effect. These results are consistent in part but not entirely with those observed by earlier investigators. The oxygen affinity of Hb M Hyde Park is between the affinity of the oxy structure and the deoxy structure of normal hemoglobin. Oxygen equilibrium curve of red cell suspension and whole hemolysate containing Hb M Hyde Park were biphasic, indicating that Hb M Hyde Park also exhibited the high oxygen affinity in those samples.

ISSN:0363-0269    

DOI:10.3109/03630268009042380

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Homoserine, asparagine and glutamine do not restore the de-formability of deoxygenated sickle cells in spite of noticeable morphological changes. These amino acids also do not raise the minimum gelling concentration of deoxyhemoglobin S. The use of these compounds as anti-sickling agents is therefore doubtful.

ISSN:0363-0269    

DOI:10.3109/03630268009042381

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Globin synthesis was measured in blood samples of four patients with hemoglobin SC Disease. In the stroma-free supernatant, globin synthesis was balanced withα/(βS+βC) = 1. In the exhaustively washed stroma, there was a preferential binding ofβchains to stroma, similar to that reported for Sickle Cell Anemia. However, while theβS/βCratio was balanced in the supernatant (0.8–0.9), theβS/βCratio of the stroma varied from 0.2 to 0.4. Therefore,βCis bound to stroma in preference toβSin hemoglobin SC disease. These findings are consistent with the concept that binding of globin to stroma is related to the electrophoretic charge of the hemoglobin.

ISSN:0363-0269    

DOI:10.3109/03630268009042382

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

A survey of hemoglobinopathies which was carried out in the Takamatsu district during the period from January to August 1979 detected six families with abnormal hemoglobins. Approximately 6010 inhabitants were screened.Three of these families had the same new Hb variant (Hb Takamatsuβ120 Lys→Gln) that has not been previously reported.Existence of a blood relationship among these three families could not be established even after careful family studies.This abnormal hemoglobin was not associated with adverse symptoms and gave normal hematologic findings in the carriers.The isopropanol test was negative, oxygen affinity was within the normal range, and biosynthetic ratio in reticulocytes was around 1.0.One of the difficulties in the structural analysis of this hemoglobin was related to complete superposition of abnormalβXT-12b,13 on aβT-8.9 peptide in the fingerprint of the trypsin digest of aminoethylated aberrantβXchain. This was overcome by collection of abnormal tryptic 3 core (βXT-10-13) from unmodifiedβXchain, and subsequent digestion by chymotrypsin. Edman analysis of the chymotryptic peptides thus obtained successfully confirmed the substitution to beβ120 Lys→Gln.

ISSN:0363-0269    

DOI:10.3109/03630268009042383

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Hemoglobin Necker Enfants-Malades was fortuitously found during a systematic measurement of Hb AIcin a diabetic patient. It displays an electrophoretic pattern similar to that of Hb A but can be distinguished by isoelectricfocusing or cation exchange chromatography. Functional studies reveal no abnormal features.

ISSN:0363-0269    

DOI:10.3109/03630268009042384

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Hemoglobin Summer Hill was found in a Lebanese woman living in Sydney, New South Wales, Australia. Its structure was shown to beβ52(D3) Asp→His. It is a stable hemoglobin which has no significant change in oxygen affinity or heme-cooperativity.

ISSN:0363-0269    

DOI:10.3109/03630268009042385

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

β-THALASSEMIA IN ASSOCIATION WITH HB LESLIE (α2β2131GLN→0)IN A SARDINIAN FAMILY

ISSN:0363-0269    

DOI:10.3109/03630268009042386

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Several lines of evidence indicate that Hb M Saskatoon (β63 His→Tyr) may be slightly unstable.In vivostudies have shown that heterozygotes for this variant have a slight b u t constant hemolysis (1-3), and, in one case, the hemolysis was markedly accelerated by the administration of a sulfonamide drug (3). In addition, Hayashi et al. have demonstratedin vitrothat Hb M Saskatoon is less stable than Hb A on exposure t o heat or to sodium benzoate (4). We have examined the biosynthesis of this variant to determine the degree of instability of the abnormalβchain. Unstableβchain variants are usually synthesized a t a normal rate. However, because they are more likely to be degraded than normalβchains, they constitute less than 50% of the totalβchain in peripheral blood and have higher specific radioactivities thanβA, as recently reviewed. (5)

ISSN:0363-0269    

DOI:10.3109/03630268009042387

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor

摘要:

Hemoglobin Mizushi is one of the hemoglobin variants detected by the hemoglobinopathy survey in Takamatsu district (1). This paper aims to describe the structural and functional characterization of this new abnormal hemoglobin.

ISSN:0363-0269    

DOI:10.3109/03630268009042388

出版商:Taylor&Francis    

年代:1980

数据来源: Taylor