Literature DB >> 604493

Haemoglobin Porto Alegre in a Cuban family.

G Martínez, F Lima, M Wade, M Estrada, B Colombo, L Heredero, H Granda.   

Abstract

During a screening programme for abnormal haemoglobins in Habana, one case of Hb Porto Alegre was found in 23 000 cases analysed. The ability of this variant to polymerise in vitro and the absence of clinical features in the carriers have been confirmed. These observations are now explained by the findings of high levels of glutathione in the red cells of subjects heterozygous for Hb Porto Alegre: it is suggested that the increase of glutathione is responsible for the absence of in vivo polymerisation and accounts for the lack of clinical symptoms.

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Year:  1977        PMID: 604493      PMCID: PMC1013638          DOI: 10.1136/jmg.14.6.422

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  11 in total

1.  An improved method for the fingerprinting of human hemoglobin.

Authors:  C BAGLIONI
Journal:  Biochim Biophys Acta       Date:  1961-04-01

2.  Hemoglobin Porto Alegre, a possible polymer of normal hemoglobin in a Caucasian Brazilian family.

Authors:  C V TONDO; F M SALZANO; D L RUCKNAGEL
Journal:  Am J Hum Genet       Date:  1963-09       Impact factor: 11.025

3.  Estimation of small percentages of foetal haemoglobin.

Authors:  K BETKE; H R MARTI; I SCHLICHT
Journal:  Nature       Date:  1959-12-12       Impact factor: 49.962

4.  An improved procedure for starch-gel electrophoresis: further variations in the serum proteins of normal individuals.

Authors:  O SMITHIES
Journal:  Biochem J       Date:  1959-03       Impact factor: 3.857

5.  Polymerization of hemoglobins of mouse and man: structural basis.

Authors:  J Bonaventura; A Riggs
Journal:  Science       Date:  1967-11-10       Impact factor: 47.728

6.  An economic high speed electrophoretic screening system for hemoglobin S and other proteins.

Authors:  L Heredero; H Granda; J A Aguiar; K Altland
Journal:  Humangenetik       Date:  1974-02-21

7.  Two more examples of Hb Porto Alegre, alpha-2 beta-2 9 Ser leads to Cys in Belém, Brazil.

Authors:  M Seid-Akhavan; M Ayres; F M Salzano; W P Winter; D L Rucknagel
Journal:  Hum Hered       Date:  1973       Impact factor: 0.444

8.  Hemoglobin Ta-Li: 83 Gly leads to Cys.

Authors:  R Q Blackwell; C S Liu; C L Wang
Journal:  Biochim Biophys Acta       Date:  1971-09-28

9.  Rapid estimation of hemoglobin A2 by DEAE chromatography.

Authors:  L F Bernini
Journal:  Biochem Genet       Date:  1969-01       Impact factor: 1.890

10.  Glutathione metabolism of the erythrocyte. The enzymic cleavage of glutathione-haemoglobin preparations by glutathione reductase.

Authors:  S K Srivastava; E Beutler
Journal:  Biochem J       Date:  1970-09       Impact factor: 3.857

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  3 in total

1.  Permanence or change? The meaning of genetic variation.

Authors:  F M Salzano
Journal:  Proc Natl Acad Sci U S A       Date:  2000-05-09       Impact factor: 11.205

2.  Asymptomatic child heterozygous for hemoglobin S and hemoglobin Pôrto Alegre.

Authors:  Liliana Lojo; Pedro Santiago-Borrero; Enid Rivera; Jessicca Renta; Carmen L Cadilla
Journal:  Pediatr Blood Cancer       Date:  2010-11-05       Impact factor: 3.167

3.  Hemoglobin Porto Alegre forms a tetramer of tetramers superstructure.

Authors:  Véronique Baudin-Creuza; Christophe Fablet; Franck Zal; Brian N Green; Danielle Promé; Michael C Marden; Josée Pagnier; Henri Wajcman
Journal:  Protein Sci       Date:  2002-01       Impact factor: 6.725

  3 in total

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