ENHANCED MG2-ATPASE ACTIVITY IN GHOSTS FROM HS ERYTHROCYTES AND IN NORMAL GHOSTS STRIPPED OF MEMBRANE SKELETAL PROTEINS MAY REFLECT ENHANCED AMINOPHOSPHOLIPID TRANSLOCASE ACTIVITY()

Hereditary spherocytosis (HS) is a congenital haemolytic anaemia which is characterized by a great variety of structural defects in the red cell's membrane skeleton and/or deficiencies in particular membrane (s keletal) proteins. Enhanced (Mg2+)-dependent adenosine triphosphatase (Mg2+-ATPase) activities, varying from 115% to 160%, were invariably f ound in erythrocyte ghosts derived from 13 HS patients. Similarly, an enhancement of Mg2+-ATPase activity by 30% is observed in normal red c ell ghosts that have been stripped of the greater part of their membra ne skeletal proteins by treatment with a low ionic strength buffer. Re association of those stripped ghosts with spectrin reduces the enhance d Mg2+-ATPase activity to its original level. Since in both cases, HS ghosts and stripped normal ghosts, the stabilizing effects that the me mbrane skeleton exerts on the maintenance of an endofacial localizatio n of the aminophospholipids are impaired, the enhanced Mg2+-ATPase act ivity is interpreted to reflect an increased activity of the aminophos pholipid translocase. The present observations therefore support a rol e of the membrane skeleton in the stabilization of phospholipid asymme try in the red cell membrane and consequently in reducing the energy c onsumption of the translocase.