Cross-linked polymers in the red cell membranes of a patient with Hb-Koln disease

Biochem Biophys Res Commun. 1987 Sep 15;147(2):602-7. doi: 10.1016/0006-291x(87)90973-9.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Centrifugation, Density Gradient
  • Chromatography, High Pressure Liquid
  • Dipeptides / blood*
  • Erythrocyte Membrane / metabolism*
  • Female
  • Hemoglobinopathies / blood*
  • Hemoglobins, Abnormal / metabolism*
  • Humans
  • Membrane Proteins / blood*
  • Polymers

Substances

  • Dipeptides
  • Hemoglobins, Abnormal
  • Membrane Proteins
  • Polymers
  • epsilon-(gamma-glutamyl)-lysine
  • hemoglobin Koln