2. MEMBRANE PROTEINS: Peripheral protein: Trophomyosin Spectrin Actin Protein 4.1 Ankyrin Protein 4.2 Integral protein: Glycophorin Band 3 protein (chloride shift) 6
7 Names Definition Function Peripheral protein Spectrin Actin 3. Ankyrin 4. Protein 4.1 5. Protein 4.2 6. Trophomyosin cytoskeletal protein that lines the intracellular side of the plasma membrane Abundant protein in cell membrane are a family of adaptor protein is a major structural element. is an ATP-binding protein Heterodimeric protein Responsible for biconcave shape of RBC participates in more protein-protein interactions Interacts with band 3 protein and spectrin to achieve linkage between bilayer and skeleton. Stabilises actin-spectrin interactions. regulate the association of protein 3 with ankyrin . Stabilizing the actin filaments.
8 Names Definition Function Integral protein Glycophorin 2. Band 3 protein Sialic acid rich glycoproteins anion exchanger 1 imparts a negative charge to the cell, reducing interaction with other cells/ endothelium Exchanges bicarbonate for chloride (chlorine shift ).
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3. CYTOSKELETON: 1. Formed by structural protein 2. Basic unit : hexagonal lattice with 6 spectrin molecules. 3. Tail end: tetramers linked to actin and protein 4.1. 4. Head end: ß spectrin linked to ankyrin PLANE OF DESIGN: 1 . Vertical interaction: • Stabilise the lipid bilayer membrane. 2. Horizontal interaction: • Maintain biconcave shape of RBC 10
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DEFECTS IN RBC CELL MEMBRANE: 1. Hereditary spherocytosis : Hereditary hemolytic disorder. (most commom ) Mutation in membrane protein (spherical rbc ) Clinical features: anemia. jaundice. fatigue. splenomegaly . 13
2. Elliptocytosis : Rare hereditary disorder. also known as ovalocytosis . Oval shape rbc . It is a hemolytic anemia (severe cases) Treatment: splenectomy 14