Impaired lysosomal function underlies monoclonal light chain–associated renal Fanconi syndrome
A Luciani, C Sirac, S Terryn, V Javaugue… - Journal of the …, 2016 - journals.lww.com
Monoclonal gammopathies are frequently complicated by kidney lesions that increase the
disease morbidity and mortality. In particular, abnormal Ig free light chains (LCs) may
accumulate within epithelial cells, causing proximal tubule (PT) dysfunction and renal
Fanconi syndrome (RFS). To investigate the mechanisms linking LC accumulation and PT
dysfunction, we used transgenic mice overexpressing human control or RFS-associated
κLCs (RFS-κLCs) and primary cultures of mouse PT cells exposed to low doses of …
disease morbidity and mortality. In particular, abnormal Ig free light chains (LCs) may
accumulate within epithelial cells, causing proximal tubule (PT) dysfunction and renal
Fanconi syndrome (RFS). To investigate the mechanisms linking LC accumulation and PT
dysfunction, we used transgenic mice overexpressing human control or RFS-associated
κLCs (RFS-κLCs) and primary cultures of mouse PT cells exposed to low doses of …