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Molecular and Cellular Biology, January 2005, p. 414-421, Vol. 25, No. 1
0270-7306/05/$08.00+0     doi:10.1128/MCB.25.1.414-421.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

WISP3, the Gene Responsible for the Human Skeletal Disease Progressive Pseudorheumatoid Dysplasia, Is Not Essential for Skeletal Function in Mice

Wendy E. Kutz,1 Yaoqin Gong,1,{dagger} and Matthew L. Warman1,2,3*

Departments of Genetics,1 Pediatrics, Case Western Reserve University,2 Center for Human Genetics, University Hospitals of Cleveland, Cleveland, Ohio3

Received 28 May 2004/ Returned for modification 14 July 2004/ Accepted 23 September 2004

In humans, loss-of-function mutations in WISP3 cause the autosomal recessive skeletal disease progressive pseudorheumatoid dysplasia (PPD) (Online Mendelian Inheritance in Man database number 208230). WISP3 encodes Wnt1-inducible signaling protein 3, a cysteine-rich, multidomain, secreted protein, whose paralogous CCN (connective tissue growth factor/cysteine-rich protein 61/nephroblastoma overexpressed) family members have been implicated in diverse biologic processes including skeletal, vascular, and neural development. To understand the role of WISP3 in the skeleton, we targeted the Wisp3 gene in mice by creating a mutant allele comparable to that which causes human disease. We also created transgenic mice that overexpress human WISP3 in cartilage. Surprisingly, homozygous Wisp3 mutant mice appear normal and do not recapitulate any of the morphological, radiographic, or histological abnormalities seen in patients with PPD. Mice that overexpress WISP3 are also normal. We conclude, that in contrast to humans, Wisp3 is not an essential participant during skeletal growth or homeostasis in mice.

* Corresponding author. Mailing address: Department of Genetics, Case Western Reserve University, 2109 Adelbert Rd., BRB 719, Cleveland, OH 44106-4955. Phone: (216) 368-4919. Fax: (216) 368-5857. E-mail: mlw14{at}po.cwru.edu.

{dagger} Present address: Shandong Medical University, Jinan, China.

Molecular and Cellular Biology, January 2005, p. 414-421, Vol. 25, No. 1
0022-538X/05/$08.00+0     doi:10.1128/MCB.25.1.414-421.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.



This article has been cited by other articles:

  • Hayes, M. J., Thomas, D., Emmons, A., Giordano, T. J., Kleer, C. G. (2008). Genetic Changes of Wnt Pathway Genes Are Common Events in Metaplastic Carcinomas of the Breast. Clin. Cancer Res. 14: 4038-4044 [Abstract] [Full Text]  
  • Leask, A., Abraham, D. J. (2006). All in the CCN family: essential matricellular signaling modulators emerge from the bunker. J. Cell Sci. 119: 4803-4810 [Abstract] [Full Text]  


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