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First published online November 3, 2003
doi: 10.1242/10.1242/dev.00861

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Development 130, 6037-6047 (2003)
Copyright © 2003 The Company of Biologists Limited

Replicated anterior zeugopod (raz): a polydactylous mouse mutant with lowered Shh signaling in the limb bud

Ottheinz Krebs1,*, Claire M. Schreiner2, William J. Scott, Jr2, Sheila M. Bell2, David J. Robbins3, John A. Goetz3, Heidi Alt1, Norm Hawes4, Eckhard Wolf1 and Jack Favor5

1 Institute of Molecular Animal Breeding and Biotechnology, Gene Center, Ludwig-Maximilian University, Munich, Germany
2 Division of Developmental Biology, Children's Hospital Research Foundation, Department of Pediatrics, University of Cincinnati, College of Medicine, Cincinnati, OH 45229, USA
3 Department of Molecular Genetics, Biochemistry and Microbiology, University of Cincinnati, College of Medicine, Cincinnati, OH 45267, USA
4 The Jackson Laboratory, Bar Harbor, ME 04609, USA
5 GSF – National Research Center for Environment and Health, Institute of Human Genetics, Neuherberg, Germany

* Author for correspondence (e-mail: krebs{at}lmb.uni-muenchen.de)

Accepted 4 September 2003

A unique limb phenotype is described in a radiation-induced mutant mouse resulting from an inversion of a proximal segment of chromosome 5. The limb phenotype in the homozygous mutant presents with two anterior skeletal elements in the zeugopod but no posterior bone, hence the name replicated anterior zeugopod, raz. The zeugopod phenotype is accompanied by symmetrical central polydactyly of hand and foot. The chromosomal inversion includes the Shh gene and the regulatory locus, located ~1 Mb away, within the Lmbr1 gene. In homozygous mutants, the expression of Shh mRNA and Shh protein is severely downregulated to about 20% of wild-type limb buds, but Shh expression appears normal throughout the remainder of the embryo. Correspondingly, Gli3 expression is upregulated and posteriorly expanded in the raz/raz limb bud. We propose that the double anterior zeugopod and symmetrical central polydactyly are due to an increased and uniform concentration of the Gli3 repressor form because of lowered Shh signaling.

Key words: Sonic hedgehog, Gli3, Central polydactyly, Radial and tibial dimelia, Mouse




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© The Company of Biologists Ltd 2003