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Related Experiment Videos

Mouse patched1 controls body size determination and limb patterning.

L Milenkovic1, L V Goodrich, K M Higgins

  • 1Departments of Developmental Biology and Genetics, Howard Hughes Medical Institute, Beckman Center, Stanford University School of Medicine, Stanford, California, USA.

Development (Cambridge, England)
|September 28, 1999
PubMed
Summary

Patched1 (Ptc1) protein normally represses Sonic hedgehog (Shh) expression. Loss of Ptc1 function leads to limb developmental abnormalities, including polydactyly, due to anterior limb bud Shh transcription.

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Area of Science:

  • Developmental biology
  • Molecular genetics
  • Genetics

Background:

  • Hedgehog (Hh) proteins are crucial for embryonic development, regulating cell fate and proliferation.
  • Patched1 (Ptc1) is an Hh-binding protein that antagonizes Hh signaling by repressing target gene transcription.
  • Sonic hedgehog (Shh) plays a key role in vertebrate limb patterning and growth, but the function of Ptc1 in later development is unclear due to early embryonic lethality in ptc1(-/-) mice.

Purpose of the Study:

  • To investigate the role of Patched1 (Ptc1) in later stages of embryonic development, particularly in limb development.
  • To understand the mechanism by which Ptc1 regulates Sonic hedgehog (Shh) expression in the limb bud.

Main Methods:

  • Generation of partially rescued ptc1 homozygous mutant mouse embryos using a metallothionein promoter driving Ptc1 expression.

Related Experiment Videos

  • Analysis of embryonic morphology, focusing on limb development and anterior digit formation.
  • Assessment of Shh expression patterns in the limb bud of wild-type and mutant embryos.

    • Main Results:

    • Transgenic Ptc1 expression in a wild-type background resulted in reduced animal size and loss of anterior digits.
    • Partially rescued ptc1 homozygotes survived to E14 with some morphological defects, including midbrain overgrowth.
    • Partially rescued embryos exhibited exencephaly, polydactyly, and branched digits, preceded by anterior limb bud Shh transcription.

    Conclusions:

    • Patched1 (Ptc1) functions to repress Sonic hedgehog (Shh) expression in the anterior limb bud.
    • Dysregulation of Ptc1 leads to developmental abnormalities in limb patterning and digit formation.
    • Ptc1 is essential for controlling Shh expression and ensuring proper limb development.