Chapter category: Tissue Engineering
Experimental Models to Study the Origin and Role of Myofibroblasts in Renal Fibrosis
Tissue Repair, Contraction
and the Myofibroblast
Edited by: Christine Chaponnier, Alexis Desmouliere and Giulio GabbianiISBN: 0-387-33649-4
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Chapter authors:
Michael Zeisberg, Mary A. Soubasakos and Raghu Kalluri
Most of the present knowledge on the pathomechanism of renal fibrosis is based on experimental studies with laboratory animals. Today, a variety of genetic and inducible animal models that mimic primary causes of human disease, such as diabetes mellitus, glomerulonephritis or lupus erythematodes are available. However, only few of these models progress consistently to interstitial fibrosis in the kidney involving interestitial fiberosis, tubular atrophy and glomerulosclerosis, common features of renal fibrogenesis. In this chapter, three different mouse models of human kidney disease are described highlighting their utility to study pathways leading to renal fibrosis. The most common diseases that cause end stage renal failure (ESRF) differ significantly in their underlying primary patho-mechanisms.1,2 Glomerulonephritis due to primary glomerular inflammation, metabolic diseases such as diabetes mellitus, cystic nephropathies such as polycystic kidney disease, interstitial nephritis due to primary interstitial inflammation, and vasculopathies are among the leading causes of end stage renal failure.1 Despite the diversity of primary patho-mechanisms associated with these different kidney diseases, they all lead to an indistinguishable scarred/fibrotic kidney.1 The observation that chronic renal failure seems to possess common patterns independent of the underlying disease, has resulted in the speculation for the existence of a common pathogenic pathway leading to ESRF associated with fibrosis.1,3 Most of the present knowledge on pathomechanism of renal fibrosis is based on experimental studies with laboratory animals. Today, a variety of genetic and inducible animal models that mimic primary causes of human disease, such as diabetes mellitus, glomerulonephritis or lupus erythematodes are available.
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