Component | Function | Clinical relevance |
Lipids | ||
PC | Major component of the outer membrane leaflet | Rare cases of hemolytic anemia with high PC |
SM | Major component of the outer membrane leaflet | Not described |
PE | Major component of the inner membrane leaflet | Not described |
PS | Major component of the inner membrane leaflet | Exposure on the outer membrane leaflet results in phagocytosis |
Cholesterol | Regulates the "flippase" that controls membrane asymmetry | Increased in liver disease, creating target cells and spur cells |
Integral membrane proteins | ||
Band 3 (AE1) | Anion exchange (bicarbonate for chloride) Links membrane to underlying cytoskeleton Carries blood groups I, Diego | Hereditary spherocytosis (HS) Southeast Asian ovalocytosis (SAO) Cryohydrocytosis (a subtype of hereditary stomatocytosis [HSt]) Distal renal tubular acidosis |
Glycophorin C | Negative charge reduces RBC-RBC and RBC-vascular endothelial interactions Links membrane to underlying cytoskeleton Carries blood groups MN, S, Gerbich | Malaria resistance |
Aquaphorin-1 | Water transport | Mild anemia due to deficiency |
Glut-1 | Glucose transport | Mild anemia due to deficiency |
Outer membrane proteins | ||
Spectrin | Major structural protein that creates hexagonal array | HS |
Ankyrin | Mechanically couples spectrin to band 3 | HS |
Band 4.2 | Strengthens ankyrin-band 3 interaction | HS |
Actin | Principal protein component of the junctional complex | Not described |
Protein 4.1 | Stabilizes spectrin-actin interaction | HS/hereditary elliptocytosis (HE) |
Adducin | Stabilizes spectrin-band 3 and spectrin-actin interactions | Not described |
آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟