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Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy.: Light-chain deposition disease

Abstract : Light-, light- and heavy-, and heavy-chain deposition diseases belong to a family of diseases that include light-chain (AL)-amyloid, nonamyloid fibrillary and immunotactoid glomerulonephritis, and cryoglobulinemic glomerulonephritis, in which monoclonal Ig or their subunits become deposited in kidney. In clinical and pathologic terms, light-, light- and heavy-, and heavy-chain deposition diseases essentially are similar and are characterized by prominent renal involvement with severe renal failure; extrarenal manifestations; diabetes-like nodular glomerulosclerosis; marked thickening of tubular basement membranes; and monotypic deposits of light chain, mostly kappa, and/or heavy chain that feature a nonorganized granular, electron-dense appearance by electron microscopy. The most common cause is myeloma. Recent progress has been made in the understanding of the molecular pathomechanisms of Ig-chain deposition and extracellular matrix accumulation, which opens up new therapeutic avenues in addition to eradication of the Ig-secreting plasma cell clone. Because these diseases represent a model of glomerular and interstitial fibrosis that is induced by a single molecule species, a better understanding of their pathomechanisms may help to unravel the pathophysiology of kidney fibrosis and renal disease progression.
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https://www.hal.inserm.fr/inserm-00169567
Contributor : Pierre Ronco <>
Submitted on : Tuesday, September 4, 2007 - 1:34:53 PM
Last modification on : Friday, May 3, 2019 - 1:42:04 AM
Long-term archiving on: : Friday, November 25, 2016 - 5:42:03 PM

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Pierre Ronco, Plaisier Emmanuelle, Mougenot Béatrice, Aucouturier Pierre. Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy.: Light-chain deposition disease. Clinical Journal of the American Society of Nephrology, American Society of Nephrology, 2006, 1 (6), pp.1342-50. ⟨10.2215/CJN.01730506⟩. ⟨inserm-00169567⟩

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