ISSN:0363-0269    

DOI:10.3109/03630267709003901

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267709003902

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

ISSN:0363-0269    

DOI:10.3109/03630267709003903

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

The trait condition for hemoglobin Tak, was found in a 4-day old newborn Malay who suffered from severe neonatal jaundice. Theβchain of the abnormal hemoglobin was elongated by 11 residues at the C-termlnus and had the same structure as reported for Hb Tak. The mother was heterozygous for this abnormal hemoglobin, the father was normal. The mother and child, 4 years later, did not show clinical or hematoiogical symptoms except definitely Increased resistance of their erythrocytes to hypotonic saline solutions and slight anisopoikilocytosis. The abnormal gene in the two reported That families and in our Malay family may have the same origin.

ISSN:0363-0269    

DOI:10.3109/03630267709003904

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

The second occurrence of Hb M Milwaukee is reported in two members of a German family who had cyanosis since early childhood. Contrary to earlier reports, Hb M Milwaukee exhibits a distinct heat instability. It is suggested, that in this family the variant resulted from a new mutation.

ISSN:0363-0269    

DOI:10.3109/03630267709003905

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

During a survey for abnormal hemoglobins in Polesine (a region north of the Po river, where betathalassemia is very frequent) a slow moving variant was noted in a 79-yr-old woman living in Gavello, a small town in the province of Rovigo. Structural studies demonstrated a previously undescribed amino acid substitution, 647 AspàGly. This new variant has been named Hb Gavello.

ISSN:0363-0269    

DOI:10.3109/03630267709003906

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

A healthy Thai male was found to have an abnormal hemoglobin moving faster than Hb A in starch-gel electrophoresis, in addition to the normal hemoglobin constituents. The abnormal hemoglobin constituted 28% of the whole hemoglobin. The subject was asymptomatic and had normal hematologic findings. Structural characterization revealed that the amino acid substitution wasα56 Lys→Thr. This variant has never been described previously and it is named Hb Thailand.

ISSN:0363-0269    

DOI:10.3109/03630267709003907

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

Hemoglobin Lufkin was found in a Black-American family. Structural analysis of the abnormal hemoglobin indicates a substitution of aspartic acid for glycine at position 29 in theβchain. Marked instability of the variant hemoglobin is demonstrated by the rapid formation of inclusion bodies upon exposure of the red cells to redox dyes and by the large percentage of precipitated hemoglobin at 65°C. The oxygen affinity, the Bohr effect, and the degree of cooperativity of Hb Lufkin and Hb A are similar over the physiologic pH range. However, at acid pH the oxygen affinity of the variant is increased. Unlike several other reported variants in the B helix, Hb Lufkin is not associated with methemoglobinemia.

ISSN:0363-0269    

DOI:10.3109/03630267709003908

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

The haem proteins can be considered, in one aspect of their function, as machines for activating oxygen. In the case of oxygen-carriers such as haemoglobin, the giobin has evolved so that its conformation limits access to the haem group, with resultant reversible release of oxygen. However, distortion of the globin may allow either the discharge of oxygen as the activated product superoxide or, more threateningly, allow direct function of the haemoglobin as an oxidative enzyme. Support for this is shown by the reaction with acetylphenylhydrazine where haemoglobin functions as both an oxidase and oxygenase. An implication of oxidase activity is the potential to initiate free radical formation particularly with unsaturated lipids. Observations of the acetylphenylhydrazine reaction emphasize the role of glutathione as a free radical scavenger.

ISSN:0363-0269    

DOI:10.3109/03630267709003909

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor

摘要:

Hb M Akita disease is an example of Hb M Hyde Park hemoglobinopathy found in Japan. It causes cyanosis and mild hemolytic anemia. A minor slow-moving red component of hemoglobin is always detected electro-phoretically together with the main chocolate-brown component of abnormal hemoglobin (Hb M Hyde Park = Hb M Akita,β92 HisàTyr). The minor component has been called Hb Akita by us since its discovery, but Hb Hyde Park or Hb HP will be the preferred name.Purified solutions of Hb HP were prepared by cellulose acetate membrane electrophoresis (pH 8.6), and its physicochemical properties were investigated in comparison with those of Hb A and Hb M Hyde Park by spectroscopy, observation of ligand reactivity and oxygen affinity (Bohr effect and the 2,3-DPG effect), starch gel electrophoresis of PCMB-treated globins, and fingerprinting of the trypsin digest of aminoethylated polypeptide chains separated by urea CMC chromatography.The results obtained revealed that (1) Hb HP possessedtheabnormalβMchain which was the same as that of Hb M Hyde Park, and (2) in the molecule of lib HP one of theβMchain pair was without heme, and (3) all of the heme irons of the normalαchains (αA) and the heme-retaining abnormalβMchain were ferrous. Accordingly, Hb HP is presumed to be a modified hemoglobin expressed by the formulaα2A(2h)βM(1h)βM(oh), where 2h, 1h and oh refer to the presence of two, one and zero hemes, respectively.

ISSN:0363-0269    

DOI:10.3109/03630267709003910

出版商:Taylor&Francis    

年代:1977

数据来源: Taylor