Goodpasture Syndrome Essay

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Goodpasture’s Syndrome Overview Goodpasture’s syndrome (Goodpasture’s disease) is a rare autoimmune disease. This disease was first identified by the American pathologist Ernest Goodpasture of Vanderbilt University in 1919. The glomerular basement membrane component of the lungs is attacked by antibodies IgG and IgM. It causes progressive glomerulonephritis and pulmonary hemorrhage if not treated on time. Collagen, Type IV, alpha 3 acts as antigen for the recognition by own antibodies and the complement is activated. Chemokines released from the activation of monocytes, neutrophils, and tissue basophils and mast cells leads to release in chemokines from these cells, which attract a further influx of neutrophils amplifying tissue injury. This disease affects about 1 in millions of population and mostly the male. Molecular Biology & Effects on the Immune System Goodpasture’s syndrome is caused by the activation of our own immune system against normal body tissues. Glomerular basement membrane (GBM) forms a barrier in our kidney and lungs whenever cells meet connective tissue. It has stable skeleton of Type IV collagen, and the alpha 3 subunit of this collagen acts as Goodpasture’s antigen. Typically these collagen molecules attach each other to form a network or basement membrane. It is positioned on chromosome 2 within base pairs 228,029,280 to 228,179,507. Furthermore, it is located between position 36 and 37 of q arm of chromosome 2. Working under normal condition this alpha 3 is not exposed for IgG or IgM antibodies to react. Antibodies bound to basement membrane attaches to Fcγreceptors which leads to activation of monocytes, neutrophils, and tissue basophils and mast cells. These cells release chemokines that attract a further influx of neutrophils amplifying the tissue injury. The autoantibodies eventually cause an activation of complement which

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