TY - JOUR
T1 - Hemolytic anemias. Failure of the red cell membrane
AU - Shohet, S. B.
AU - Ness, P. M.
PY - 1976
Y1 - 1976
N2 - The normal erythrocyte membrane is composed of nearly equivalent amounts of lipid and protein. The lipid portion of the membrane has been well studied. Even though de novo synthesis of lipid does not occur in human red cells, many biochemical pathways exist which facilitate detoxification of lipid breakdown products and lipid renewal. Rare defects in these processes are associated with hemolytic disorders. Recent studies have revealed that the membrane proteins are diverse and suggest that protein dysfunction may also account for clinical disease. Protein and lipid are entwined in a physicochemical relationship which is probably best depicted by the classic lipid bilayer with interspersed proteins in both the inner and outer surfaces and also spanning the bilayer. Membrane failure results in hemolytic anemia. This failure can be intrinsic, caused by abnormal lipid or protein constituents; or extrinsic, with a normal membrane being unable to counteract physical, chemical, or immunologic stress. Clinical examples of membrane failure and hemolytic anemia can be separated into 3 groups according to the predominant mechanism of the hemolysis: fragmentation, whole cell lysis, and filtration and entrapment. Although these mechanisms can act separately or in concert, the final hemolytic destruction of the cell can usually be traced to a failure of membrane function.
AB - The normal erythrocyte membrane is composed of nearly equivalent amounts of lipid and protein. The lipid portion of the membrane has been well studied. Even though de novo synthesis of lipid does not occur in human red cells, many biochemical pathways exist which facilitate detoxification of lipid breakdown products and lipid renewal. Rare defects in these processes are associated with hemolytic disorders. Recent studies have revealed that the membrane proteins are diverse and suggest that protein dysfunction may also account for clinical disease. Protein and lipid are entwined in a physicochemical relationship which is probably best depicted by the classic lipid bilayer with interspersed proteins in both the inner and outer surfaces and also spanning the bilayer. Membrane failure results in hemolytic anemia. This failure can be intrinsic, caused by abnormal lipid or protein constituents; or extrinsic, with a normal membrane being unable to counteract physical, chemical, or immunologic stress. Clinical examples of membrane failure and hemolytic anemia can be separated into 3 groups according to the predominant mechanism of the hemolysis: fragmentation, whole cell lysis, and filtration and entrapment. Although these mechanisms can act separately or in concert, the final hemolytic destruction of the cell can usually be traced to a failure of membrane function.
UR - http://www.scopus.com/inward/record.url?scp=0017092722&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017092722&partnerID=8YFLogxK
U2 - 10.1016/S0025-7125(16)31839-9
DO - 10.1016/S0025-7125(16)31839-9
M3 - Article
C2 - 181647
AN - SCOPUS:0017092722
SN - 0025-7125
VL - 60
SP - 913
EP - 932
JO - Medical Clinics of North America
JF - Medical Clinics of North America
IS - 5
ER -