Red Blood Cell Membrane Flashcards
STRUCTURE, FUNCTIONS AND DEFECTS
The Red Blood Cell characteristics
7-8μm in diameter
Flexible biconcave
Disc shaped
Anuclear
Central area of pallor
Enclosed in a thin membrane called the RED BLOOD CELL MEMBRANE
RED BLOOD CELL MEMBRANE components and %
LIPIDS 40% - Phospholipids (20%), - Cholesterol (20%)
CARBOHYDRATES 10%
PROTEINS 50%
3 Major structural elements
Lipid bilayer
Integral Proteins
Membrane Skeleton
Lipid Bilayer consists of
Phospholipids
Cholesterol
Integral Proteins consist of
Band 3
Glycophorins
Membrane Skeleton
Spectrin
Ankyrin
Protein 4.1/4.2
P55
Actin
Adducin
What are the VERTICAL INTERACTIONS that make up the rbc membrane?
Ankyrin
α/β spectrin
Band 3
Protein 4.2
HORIZONTAL INTERACTION
α/β spectrin
Ankyrin
Protein 4.1
Lipid bilayer functions
Forms a matrix in which membrane proteins reside
Aids selective permeability of substances into and out of the cell
Plays a role in red cell adhesion to endothelium
Functions of carbohydrates
Carbohydrates
Serve as blood group antigens
Functions of membrane proteins
A. Integral
Band 3 acts as an anion exchange protein – Allows RBCs to exchange Cl- for HCO3-
Transport of CO2 from tissues to the lungs
B. Cytoskeleton
Determine RBC integrity, shape and flexibility (deformability)
Membranopathies characteristics and examples
Mostly hereditary disorders
Cause haemolytic anaemias
Hereditary spherocytosis
Hereditary elliptocytosis
South-East Asian ovalocytosis
Hereditary stomatocytosis
Hereditary pyro-poikilocytosis
HEREDITARY SPHEROCYTOSIS (HS) unique quality
Most common among the membranopathies
Most common cause of haemolytic anaemia among Northern Europeans
Aetio-pathogenesis of HEREDITARY SPHEROCYTOSIS (HS) - Cause
Caused by defects in the proteins responsible for the VERTICAL INTERACTION
Autosomal Dominant (rarely may be autosomal recessive)
RBCs progressively loose
membrane (loss of surface area relative to volume)
The red cells consequently appear spherical instead of biconcave disc shaped
Spherocytes are not flexible, thus cannot pass through the micro-circulation
Consequently, they are destroyed by the spleen
Clinical features of HEREDITARY SPHEROCYTOSIS (HS)
Symptoms and signs of anaemia (can present at any age)
Jaundice
Epigastric pain (due to pigment gallstones)
Splenomegaly