Goose barnacle
Goodpasture antigen is a protein found in the basement membrane of the kidney and lung. It is named after Dr. Ernest Goodpasture, who first described the associated disease, Goodpasture syndrome, in 1919.
Structure and Function
The Goodpasture antigen is a component of the type IV collagen family, specifically the α3(IV)NC1 and α5(IV)NC1 isoforms. These proteins are crucial for the structural integrity of basement membranes in various tissues, including the kidneys and lungs.
Role in Disease
In some individuals, the immune system mistakenly identifies the Goodpasture antigen as a foreign substance, leading to the production of autoantibodies. These autoantibodies attack the basement membranes in the kidneys and lungs, resulting in Goodpasture syndrome. This rare but serious condition can cause kidney failure and lung damage.
Diagnosis and Treatment
The presence of autoantibodies against the Goodpasture antigen can be detected through a blood test. This is often used in the diagnosis of Goodpasture syndrome. Treatment typically involves suppressing the immune response with medications such as corticosteroids and cyclophosphamide, and in severe cases, plasma exchange may be used to remove the harmful autoantibodies from the blood.
Research
Research into the Goodpasture antigen and its role in disease is ongoing. Understanding why the immune system targets this protein in some individuals but not others could lead to new treatments for Goodpasture syndrome and other autoimmune diseases.
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Contributors: Prab R. Tumpati, MD