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Zebrafish Provide Insight Into Human Joint Disease

Date:
September 10, 2007
Source:
Journal of Clinical Investigation
Summary:
Progressive pseudorheumatoid dysplasia (PPD), which causes joint failure in early adulthood, results from genetic mutations in the WISP3 gene that lead to the generation of nonfunctional WISP3 protein. Understanding why WISP3 deficiency causes PPD has been difficult because mice lacking WISP3 have no apparent symptoms. In a new study, scientists provide insight into the functions of WISP3 by studying its function in zebrafish.
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Progressive pseudorheumatoid dysplasia (PPD), which causes joint failure in early adulthood, results from genetic mutations in the WISP3 gene that lead to the generation of nonfunctional WISP3 protein.

Understanding why WISP3 deficiency causes PPD has been difficult because mice lacking WISP3 have no apparent symptoms. In a new study, Matthew Warman and colleagues from Case Western Reserve University, Cleveland, provide insight into the functions of WISP3 by studying its function in zebrafish.

It was shown that overexpression of WISP3 in zebrafish inhibited signaling through two proteins -- BMP and Wnt. Zebrafish and human WISP3 were also shown to inhibit BMP and Wnt signaling in mammalian cells.

Importantly, WISP3 resembling that found in individuals with PPD was not as effective at inhibiting BMP and Wnt signaling.

As cartilage development in zebrafish in which WISP3 expression was eliminated was defective, the authors conclude that dysregulated BMP and/or Wnt signaling contributes to the cartilage defects, and thereby joint failure, observed in individuals with PPD.

Article: The CCN family member Wisp3, mutant in progressive pseudorheumatoid dysplasia, modulates BMP and Wnt signaling, The Journal of Clinical Investigation


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Journal of Clinical Investigation. "Zebrafish Provide Insight Into Human Joint Disease." ScienceDaily. ScienceDaily, 10 September 2007. <www.sciencedaily.com/releases/2007/09/070907110144.htm>.
Journal of Clinical Investigation. (2007, September 10). Zebrafish Provide Insight Into Human Joint Disease. ScienceDaily. Retrieved May 23, 2017 from www.sciencedaily.com/releases/2007/09/070907110144.htm
Journal of Clinical Investigation. "Zebrafish Provide Insight Into Human Joint Disease." ScienceDaily. www.sciencedaily.com/releases/2007/09/070907110144.htm (accessed May 23, 2017).

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