Article
Molecular mechanism of amyloidogenic mutations in hypervariable regions of antibody light chains.
The Journal of biological chemistry - 1 Jan 2000
Rottenaicher Georg J, Weber Benedikt, Rührnößl Florian, Kazman Pamina, Absmeier Ramona M, Hitzenberger Manuel, Zacharias Martin, Buchner Johannes
Abstract excerpt
Systemic light chain (AL) amyloidosis is a fatal protein misfolding disease in which excessive secretion, misfolding, and subsequent aggregation of free antibody light chains eventually lead to deposition of amyloid plaques in various organs. Patient-specific mutations in the antibody VL domain are closely linked to the disease, but the molecular mechanisms by which certain mutations induce misfolding and amyloid...
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