Abstract:
:CBP is a transcriptional coactivator required by many transcription factors for transactivation. Rubinstein-Taybi syndrome, which is an autosomal dominant syndrome characterized by abnormal pattern formation, has been shown to be associated with mutations in the Cbp gene. Furthermore, Drosophila CBP is required in hedgehog signaling for the expression of decapentapleigic, the Drosophila homologue of bone morphogenetic protein. However, no direct evidence exists to indicate that loss of one copy of the mammalian Cbp gene affects pattern formation. Here, we show that various abnormalities occur at high frequency in the skeletal system of heterozygous Cbp-deficient mice resulting from a C57BL/6-CBA x BALB/c cross. In support of a conserved signaling pathway for pattern formation in insects and mammals, the expression of Bmp7 was found to be reduced in the heterozygous mutants. The frequency of the different abnormalities was significantly lower in a C57BL/6-CBA background, suggesting that the genetic background is an important determinant of the variability and severity of the anomalies seen in Rubinstein-Taybi syndrome patients.
journal_name
Proc Natl Acad Sci U S Aauthors
Tanaka Y,Naruse I,Maekawa T,Masuya H,Shiroishi T,Ishii Sdoi
10.1073/pnas.94.19.10215subject
Has Abstractpub_date
1997-09-16 00:00:00pages
10215-20issue
19eissn
0027-8424issn
1091-6490journal_volume
94pub_type
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