Hemoglobin Debrousse (beta 96[FG3]Leu-->Pro): a new unstable hemoglobin with twofold increased oxygen affinity.

Abstract

Hemoglobin Debrousse (beta 96[FG3]Leu-->Pro) is a new unstable variant, with high oxygen affinity responsible, in the steady state, for an apparently well-compensated chronic hemolytic anemia. The functional properties of this variant are due to the replacement of a leucine residue which is involved in the hydrophobic environment of the proximal side of the heme. This electrophoretically neutral hemoglobin was found as a de novo case in a 6-year-old girl suffering from severe anemia with hemolysis and transient aplastic crisis, following infection by parvovirus B19.

• Authors: P Lacan, J Kister, A Francina, G Souillet, F Galactéros, J Delaunay, H Wajcman
• Journal: American journal of hematology (Am J Hematol) Vol. 51 Issue 4 Pg. 276-81 (Apr 1996) ISSN: 0361-8609 [Print] United States
• PMID: 8602627 (Publication Type: Case Reports, Journal Article)

Chemical References

• Hemoglobins
• Hemoglobins, Abnormal
• Oxyhemoglobins
• hemoglobin Debrousse
• Globins
• deoxyhemoglobin
• Oxygen

Topics

• Adult
• Amino Acid Sequence
• Anemia, Aplastic (etiology)
• Anemia, Hemolytic, Congenital (complications, genetics)
• Base Sequence
• Blood Protein Electrophoresis
• Child
• DNA Mutational Analysis
• Erythema Infectiosum (complications)
• Female
• Globins (genetics)
• Hemoglobins
• Hemoglobins, Abnormal (genetics, isolation & purification, metabolism)
• Humans
• Leukocytes (chemistry)
• Male
• Models, Molecular
• Molecular Sequence Data
• Oxygen (blood)
• Oxyhemoglobins (metabolism)
• Point Mutation
• Polymerase Chain Reaction
• Protein Conformation
• Spermatozoa (chemistry)