AL Amyloidosis (Amyloidosis Antibody Light). Part 1. Definition, Classification, Amyloid Structure, Development and Etiopathogenesis of AL Amyloidosis

Amyloidosis is an inherited or acquired systemic storage disease in which a pathologic, amorphous substance produced as a result of abnormal protein metabolism and resistant to proteolysis is deposited in the extracellular space of various tissues (intracellular deposits occur rarely). This leads to the destruction of normal tissue architecture and disturbs its function. The development of amyloidosis is associated with the transformation of the alpha-spiral conformation of fibrous protein into a stratified, parallel, folded spatial beta-conformation. The transition of the fibrous proteins conformation and their tissue localization causes resistance of the fibers to proteolytic processes as a result of a limited access of the protein converting enzymes. In this paper we show the actual classification of amyloidosis and up-to-date knowledge about the amyloid structure, development and etiopathogenesis of AL amyloidosis (Adv Clin Exp Med 2011, 20, 5, 647-652).