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Article

Role of the monoclonal chain V domain and reversibility of renal damage in a transgenic model of acquired Fanconi syndrome

16 Mar 2006

Abstract excerpt

Acquired Fanconi syndrome (FS) is a complication of monoclonal gammopathies featuring a generalized dysfunction of the proximal tubule of the kidney, due to the storage within proximal tubular cells of a monoclonal immunoglobulin light chain. We engineered transgenic mice in which the endogenous mouse Jkappa cluster was replaced by a human VkappaJkappa rearranged gene cloned from a patient with smoldering...

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PMID: 16543471
DOI: 10.1182/blood-2005-11-4419

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