Deficient Red-Cell Spectrin in Severe, Recessively Inherited Spherocytosis

Peter Agre, Eugene P. Orringer, Vann Bennett

Research output: Contribution to journalArticlepeer-review

127 Scopus citations


RED-CELL-membrane architecture has been extensively studied in recent years.1,2 When the cells are hypotonically lysed and washed free of hemoglobin, the resulting membrane ghosts consist of the lipid bilayer and associated proteins. When ghosts are extracted with non-ionic detergents, lipid and integral membrane proteins are removed, leaving a cytoskeleton3 that is thought to be responsible for the shape, strength, and reversible deformability of the cell. The cytoskeleton consists of an assembly of polypeptides: spectrin (the largest and most abundant protein of the membrane), band 4.1, and actin. The cytoskeleton is attached to the membrane by the association of spectrin with.

Original languageEnglish (US)
Pages (from-to)1155-1161
Number of pages7
JournalNew England Journal of Medicine
Issue number19
StatePublished - May 13 1982

ASJC Scopus subject areas

  • Medicine(all)


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