Red cell membrane transport abnormalities.

Abstract

PURPOSE OF REVIEW:

The present review describes the red cell transport abnormalities of proteins of the band 3 macrocomplex. The macrocomplex is involved in red cell gas exchange and recent findings have furthered our understanding of this process.

RECENT FINDINGS:

Study of a novel band 3 hereditary spherocytosis variant suggests that expression of mutant band 3 protein can be rescued by wild-type band 3. Other studies show that some mutant band 3 protein can mediate a cation conductance. Recent work suggests both Rh-associated glycoprotein and aquaporin act as gas channels confirming the integrated function of the macrocomplex and the importance of its role in red cell gas transport.

SUMMARY:

The most recent studies on band 3-induced hereditary spherocytosis are reviewed and an explanation for the mild phenotype of heterozygous hereditary spherocytosis is discussed. A number of red cell conditions (hereditary stomatocytosis, south-east Asian ovalocytosis, distal renal tubular acidosis, Rhnull), associated with both stomatocytosis and a cation leak, are described. The evidence that Rh-associated glycoprotein forms a gas channel that transports CO2 and/or NH3 is reviewed and discussed, together with recent studies that show that aquaporin 1 transports both CO2 and O2.

• Authors: Lesley J Bruce
• Journal: Current opinion in hematology (Curr Opin Hematol) Vol. 15 Issue 3 Pg. 184-90 (May 2008) ISSN: 1065-6251 [Print] United States
• PMID: 18391782 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Anion Exchange Protein 1, Erythrocyte
• Blood Proteins
• Membrane Glycoproteins
• RHAG protein, human
• Carbon Dioxide
• Aquaporin 1
• Oxygen

Topics

• Acidosis, Renal Tubular (genetics)
• Anemia, Hemolytic (genetics)
• Anion Exchange Protein 1, Erythrocyte (physiology)
• Aquaporin 1 (physiology)
• Blood Proteins (physiology)
• Carbon Dioxide (metabolism)
• Erythrocyte Membrane (physiology)
• Humans
• Membrane Glycoproteins (physiology)
• Oxygen (metabolism)
• Spherocytosis, Hereditary (physiopathology)