Current opinion in hematology · 2008
Red cell membrane transport abnormalities.
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Bruce Lesley J
Аннотация
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.
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.