An in vitro model of light chain deposition disease

Abstract:

Nodular glomerulosclerosis results from increased deposition of extracellular matrix proteins and monotypic light chains. The inability of mesangial cells to degrade abnormal levels of tenascin-C—along with the increased expression of some growth factors such as platelet-derived growth factor (PDGF) and transforming growth factor-β (TGF-β)—is crucial to the pathogenesis of light chain deposition disease (LCDD). In order to study the molecular processes contributing to LCDD, we grew mesangial cells in three-dimensional matrices and incubated the cells with free light chains purified from the urine of patients with biopsy-proven LCDD, immunoglobulin-associated amyloid deposits, or myeloma cast nephropathy. Light chains of the latter two cohorts served as controls. Mesangial cells incubated with light chains from patients with LCDD show a significant increase in tenascin-C expression, centrally located within …

Año de publicación:

2009

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Fuente:

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