Publication: Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome
Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome
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Luciani, A., Sirac, C., Terryn, S., Javaugue, V., Prange, J. A., Bender, S., Bonaud, A., Cogné, M., Aucouturier, P., Ronco, P., Bridoux, F., & Devuyst, O. (2016). Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome. Journal of the American Society of Nephrology (JASN), 27(7), 2049–2061. https://doi.org/10.1681/ASN.2015050581
Abstract
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Abstract
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 corresponding human κLCs (25 μ
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- Luciani, Alessandro
- Sirac, Christophe
- Terryn, Sara
- Javaugue, Vincent
- Prange, Jenny Ann
- Bender, Sébastien
- Bonaud, Amélie
- Cogné, Michel
- Aucouturier, Pierre
- Ronco, Pierre
- Bridoux, Frank
- Devuyst, Olivier
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Luciani, A., Sirac, C., Terryn, S., Javaugue, V., Prange, J. A., Bender, S., Bonaud, A., Cogné, M., Aucouturier, P., Ronco, P., Bridoux, F., & Devuyst, O. (2016). Impaired Lysosomal Function Underlies Monoclonal Light Chain-Associated Renal Fanconi Syndrome. Journal of the American Society of Nephrology (JASN), 27(7), 2049–2061. https://doi.org/10.1681/ASN.2015050581