Cross-linked polymers in the red cell membranes of a patient with Hb-Koln disease.
Membranes isolated from the red cells of a Hb-Koln patient were shown to contain appreciable amounts of N epsilon-(gamma-glutamyl)lysine, the characteristic product of the post-translational cross-linking of proteins by transglutaminase. Such an apparently irreversible fusion of membrane proteins may contribute to the membrane defect in Hb-Koln disease.
D000328:Adult; D002499:Centrifugation, Density Gradient; D002851:Chromatography, High Pressure Liquid; D004151:Dipeptides / Q000097:blood*; D004910:Erythrocyte Membrane / Q000378:metabolism*; D005260:Female; D006453:Hemoglobinopathies / Q000097:blood*; D006455:Hemoglobins, Abnormal / Q000378:metabolism*; D006801:Humans; D008565:Membrane Proteins / Q000097:blood*; D011108:Polymers
D004151:Dipeptides; D006455:Hemoglobins, Abnormal; D008565:Membrane Proteins; D011108:Polymers; C001299:epsilon-(gamma-glutamyl)-lysine; C034409:hemoglobin Koln