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This article discusses the effects of abnormalities in cell membrane fluidity, specifically focusing on decreased erythrocyte membrane fluidity. It explores the causes of decreased fluidity and provides an example of a disease, Bassen-Kornzweig syndrome, that leads to altered lipid composition and reduced fluidity in red blood cell membranes. Understanding these abnormalities is important as they can have significant implications for cell function and overall health.
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BiochemistryDVT 1094 Abdul Muhaimin b. AbdWahab D11 A007Azwan b. Kamarudin D11 B038
Questions • Discuss the effect of abnormalities of cell membrane fluidity. - AzwanKamarudin - • State example of disease and explain how the disease develop. - Abdul MuhaiminAbdWahab -
Membrane Fluidity In biology, the membrane fluidity refers to the viscosity of the lipid bilayer of a cell membrane. The membrane phospholipids incorporate fatty acids of varying length and saturation. Shorter-chain fatty acids, and ones with greater unsaturation, are less stiff, less viscous and have lower melting points.
Abnormalities of Membrane Fluidity Decreased erythrocyte membrane fluidity What causes ? Due to what disease ? Decreased lipids membrane fluidity In what cell ? What causes ? What is Bassen-Kornzweig syndrome ?
1. Decreased erythrocyte membrane fluidity and altered lipid composition in human liver disease. • Changes in erythrocyte membrane lipid composition associated with abnormalplasma lipoproteins in liver disease patient. • Cholesterol/phospholipid and phosphatidylcholine/sphingomyeli molar ratios are increased. • Phospholipid fatty acid composition is abnormal. • Proportion of arachidonic acid, stearic acid are decreased while palmitic acid raised.
1. Decreased erythrocyte membrane fluidity and altered lipid composition in human liver disease. • Cause membrane fluidity in patient erythrocytes decrease and fluidity of lipid extracts from membranes patient erythrocytes also decrease. • Decrease membrane fluidity is MAIN consequence of altered lipid composition rather than protein abnormalities.
1. Decreased erythrocyte membrane fluidity and altered lipid composition in human liver disease. • In liver disease, changes in composition of phospholipidbilayer matrix in erythrocytes membrane has little influence on its fluidity. • Reduced fluidity predominantly result of increased in cholesterol relative to phospholipid.
2. Decreased Fluidity of Red Cell Membrane Lipids in Abetalipoproteinemia • Acanthocytic red cells in patients with abetalipoproteinemia (Bassen-Kornzweigsyndrom) are morphologically similar to red cells in spur cell anemia. • Bassen-Kornzweig syndrome is a rare disease passed down through families in which a person is unable to fully absorb dietary fats through the intestines.. • Decrease in fluidity of membrane lipids is due to excess cholesterol content. • Acanthocyte membranes have an increased content of sphingomyelin and a decreased content of lecithin.
2. Decreased Fluidity of Red Cell Membrane Lipids in Abetalipoproteinemia • Increased sphingomyelin/lecithin ratio of acanthocytes is responsible for their decreased membrane fluidity. • As in spur cells and in red cells enriched with cholesterol in vitro, this decrease in membrane fluidity occurs coincidentally with an abnormality in cell contour and an impairment in cell deformability.
Spiculated thorny RBCs (acanthocytes) in abetalipoproteinemia individual Acanthocytes in individual with spur cell hemolytic anemia.
Summary • Liver disease and Bassen-Kornzweig syndrome are the disease that cause decrease fluidity in cell membrane of RBCs. • Due to increase of lipid composition. • Less permeability to water and other small hydrophilic molecule . • Abnormalities in membrane fluidity lead to many disadvantages.