RBC AND DISORDERS 1 Flashcards
Factors for normal erythrocyte survival and function
- RBC membrane
- Hemoglobin Structure
- Metabolic pathways
Red Cell Membrane Structure
Membrane : Trilaminar structure (dark-light-dark band arrangement of layers)
- Outer hydrophilic portion
- glycolipid, glycoprotein & protein - Central hydrophobic portion
- protein, cholesterol and phospholipid - Inner hydrophilic portion
- protein
Principal structural element of RBC membrane; plays a major role in the RBC
cytoskeletal membrane organization (membrane integrity)
Spectrin
Primary determinant of primary mechanical coupling of the lipid bilayer to the membrane skeleton (membrane integrity)
Ankyrin
Promotes the association of spectrin with F-actin in a manner similar to band 4.1
Adducin
Dual function:
1. Promotes high affinity association between spectrin and F actin
2. Link the skeleton to the membrane by virtue of its associations with glycophorin
and band 3
Band 4.1
Catalyzes chloride-bicarbinate exchange; contains binding sites for ankyrin, band 4.1,
band 4.2 and several glycolytic enzymes; play a role in recognition and removal of
normal senescent RBCs
Band 3
Unknown function; deficiency is associated with several hemolytic anemias
Band 4.2
Transport negatively charged sialic acid
Glycoporins
Enzyme that converts protophorphyrin IX and Iron in ferrous form to Heme
Ferrochelatase
• Occurs in the cytoplasm of the normoblast and reticulocyte
- Polypeptide chains are manufactured in the ribosomes
- Made through transcription of the genetic code to messenger RNA and
translation of mRNA to the globin polypeptide chain
Globin Synthesis
Six structural genes control the synthesis of globin chains:
- Chromosome 16 : α, ζ
- Chromosome 11 : γ, β, δ, ε
Synthesis is stimulated by tissue hypoxia (decreased Oxygen)
Hemoglobin
Hemoglobin Reference
- Men
- Women
- Newborn
Men: 14-18 g/dL
Women: 12-15g/dL
Newborn: 16.5 - 21.5 g/dL
– glycoprotein that stimulates proliferation and differentiation of erythrocytes
– produced primarily in the kidney; also liver
– responds to hypoxia
Erythropoietin (EPO)
Rate Key rate limiting enzyme of Heme
α-Aminolevulinate synthase (ALAS)
+ Production is regulated by the rate at which DNA is transcribed tomRNA
+ Amount of the specific globin synthesized = to the content of their individual globin mRNAS
- α-Chain contains 141 amino acids
- β-Chain contains 146 amino acids
globin
Functions:
1. Binds oxygen readily in the lung and transport it to the peripheral tissues.
– 1 Hemoglobin molecule carry up to 4 Oxygen molecule
– 1.34 ml O2 is bound to each gram of Hemoglobin
– Red cell mass of adult contain approximately 600 grams of hemoglobin, capable of carrying 800 ml of O2
2. Transport of carbon dioxide from the peripheral tissues to the lung for excretion.
Hb
Embden-Meyerhof Pathway
Anaerobic Glycolysis