title: Mesangial cells as amyloid factory: a unique contribution of animal models.
abstract: AL amyloidosis is a severe complication of plasma-cell disorders, secondary to monoclonal immunoglobulin light chain (LC) deposition in the kidney and other organs. Though the physicochemical properties of amyloid-forming monoclonal LCs have been demonstrated to be involved in their propensity to aggregate, it remains unclear where, when, and finally why amyloid fibrils are formed in vivo. Teng et al. shed light on this long-standing issue thanks to a new animal model.
authors.affiliation: 1] University of Limoges, CNRS UMR 7276, Immunology, Limoges, France [2] National Reference Center for AL Amyloidosis and Other Monoclonal Immunoglobulin Deposition Diseases, Limoges, Poitiers, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Sirac
authors.affiliation: 1] National Reference Center for AL Amyloidosis and Other Monoclonal Immunoglobulin Deposition Diseases, Limoges, Poitiers, France [2] University Hospital La Milétrie, University of Poitiers, Department of Nephrology and Kidney Transplantation, Poitiers, France.
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
mesh_terms: D000682:Amyloid / Q000096:biosynthesis*; D000686:Amyloidosis / Q000378:metabolism*; D000818:Animals; D004195:Disease Models, Animal; D005920:Glomerular Mesangium / Q000378:metabolism*; D007147:Immunoglobulin Light Chains / Q000494:pharmacology*; D007674:Kidney Diseases / Q000378:metabolism*; D008247:Lysosomes / Q000502:physiology*; D008297:Male
chemical_list: D000682:Amyloid; D007147:Immunoglobulin Light Chains