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Science 5 June 1998:
Vol. 280. no. 5369, pp. 1603 - 1607
DOI: 10.1126/science.280.5369.1603
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Reports

Teratogen-Mediated Inhibition of Target Tissue Response to Shh Signaling

Michael K. Cooper, Jeffery A. Porter, * Keith E. Young, Philip A. Beachy dagger

Veratrum alkaloids and distal inhibitors of cholesterol biosynthesis have been studied for more than 30 years as potent teratogens capable of inducing cyclopia and other birth defects. Here, it is shown that these compounds specifically block the Sonic hedgehog (Shh) signaling pathway. These teratogens did not prevent the sterol modification of Shh during autoprocessing but rather inhibited the response of target tissues to Shh, possibly acting through the sterol sensing domain within the Patched protein regulator of Shh response.

M. K. Cooper, Department of Neurology and Howard Hughes Medical Institute, Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
J. A. Porter, K. E. Young, P. A. Beachy, Howard Hughes Medical Institute, Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
*   Present address: Ontogeny Incorporated, 45 Moulton Street, Cambridge, MA 02138.

dagger    To whom correspondence should be addressed.

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N. Fuse, T. Maiti, B. Wang, J. A. Porter, T. M. T. Hall, D. J. Leahy, and P. A. Beachy (1999)
PNAS 96, 10992-10999
   Abstract »    Full Text »    PDF »
Energetics and Topology of CzcA, a Cation/Proton Antiporter of the Resistance-Nodulation-Cell Division Protein Family.
M. Goldberg, T. Pribyl, S. Juhnke, and D. H. Nies (1999)
J. Biol. Chem. 274, 26065-26070
   Abstract »    Full Text »    PDF »
Marked Alteration of Sterol Metabolism and Composition without Compromising Retinal Development or Function.
S. J. Fliesler, M. J. Richards, C.-y. Miller, and N. S. Peachey (1999)
Invest. Ophthalmol. Vis. Sci. 40, 1792-1801
   Abstract »    Full Text »    PDF »
Cholesterol Biosynthesis from Lanosterol. MOLECULAR CLONING, TISSUE DISTRIBUTION, EXPRESSION, CHROMOSOMAL LOCALIZATION, AND REGULATION OF RAT 7-DEHYDROCHOLESTEROL REDUCTASE, A SMITH-LEMLI-OPITZ SYNDROME-RELATED PROTEIN.
S.-H. Bae, J. N. Lee, B. U. Fitzky, J. Seong, and Y.-K. Paik (1999)
J. Biol. Chem. 274, 14624-14631
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Left-Right Asymmetry and Kinesin Superfamily Protein KIF3A: New Insights in Determination of Laterality and Mesoderm Induction by kif3A-/- Mice Analysis.
S. Takeda, Y. Yonekawa, Y. Tanaka, Y. Okada, S. Nonaka, and N. Hirokawa (1999)
J. Cell Biol. 145, 825-836
   Abstract »    Full Text »    PDF »
Association of Sterol- and Glycosylphosphatidylinositol-linked Proteins with Drosophila Raft Lipid Microdomains.
A. Rietveld, S. Neutz, K. Simons, and S. Eaton (1999)
J. Biol. Chem. 274, 12049-12054
   Abstract »    Full Text »    PDF »


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