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Transposon mutagenesis with coat color genotyping identifies an essential role for Skor2 in sonic hedgehog signaling and cerebellum development.

http://www.ncbi.nlm.nih.gov/pubmed/21937600

Correct development of the cerebellum requires coordinated sonic hedgehog (Shh) signaling from Purkinje to granule cells. How Shh expression is regulated in Purkinje cells is poorly understood. Using a novel tyrosinase minigene-tagged Sleeping Beauty transposon-mediated mutagenesis, which allows for coat color-based genotyping, we created mice in which the Ski/Sno family transcriptional co-repressor 2 (Skor2) gene is deleted. Loss of Skor2 leads to defective Purkinje cell development, a severe reduction of granule cell proliferation and a malformed cerebellum. Skor2 is specifically expressed in Purkinje cells in the brain, where it is required for proper expression of Shh. Skor2 overexpression suppresses BMP signaling in an HDAC-dependent manner and stimulates Shh promoter activity, suggesting that Skor2 represses BMP signaling to activate Shh expression. Our study identifies an essential function for Skor2 as a novel transcriptional regulator in Purkinje cells that acts upstream of Shh during cerebellum development.

Pubmed ID: 21937600 RIS Download

Mesh terms: Animals | Bone Morphogenetic Proteins | Cell Differentiation | Cerebellum | Gene Expression Regulation, Developmental | Genotype | Hair Color | Hedgehog Proteins | Histone Deacetylases | Mice | Mice, Knockout | Mice, Transgenic | Monophenol Monooxygenase | Mutagenesis, Insertional | Proto-Oncogene Proteins | Purkinje Cells | Repressor Proteins | Signal Transduction | Transforming Growth Factor beta | Transposases

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Associated grants

  • Agency: NIA NIH HHS, Id: AG020670
  • Agency: NIA NIH HHS, Id: AG032051
  • Agency: NICHD NIH HHS, Id: HD024064

Mouse Genome Informatics (Data, Gene Annotation)

Expression Atlas of the Marmoset (Data, Gene Annotation)

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