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PMID:12446761
| Citation |
Kim, SW, Cheong, C, Sohn, YC, Goo, YH, Oh, WJ, Park, JH, Joe, SY, Kang, HS, Kim, DK, Kee, C, Lee, JW and Lee, HW (2002) Multiple developmental defects derived from impaired recruitment of ASC-2 to nuclear receptors in mice: implication for posterior lenticonus with cataract. Mol. Cell. Biol. 22:8409-14 |
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| Abstract |
ASC-2, a recently isolated transcriptional coactivator molecule, stimulates transactivation by multiple transcription factors, including nuclear receptors. We generated a potent dominant negative fragment of ASC-2, encompassing the N-terminal LXXLL motif that binds a broad range of nuclear receptors. This fragment, termed DN1, specifically inhibited endogenous ASC-2 from binding these receptors in vivo, whereas DN1/m, in which the LXXLL motif was mutated to LXXAA to abolish the receptor interactions, was inert. Interestingly, DN1 transgenic mice but not DN1/m transgenic mice exhibited severe microphthalmia and posterior lenticonus with cataract as well as a variety of pathophysiological phenotypes in many other organs. Our results provide a novel insight into the molecular and histopathological mechanism of posterior lenticonus with cataract and attest to the importance of ASC-2 as a pivotal transcriptional coactivator of nuclear receptors in vivo. |
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| Keywords |
Amino Acid Motifs; Animals; Cells, Cultured; Congenital Abnormalities; Disease Models, Animal; Embryo, Mammalian/anatomy & histology; Embryo, Mammalian/pathology; Embryo, Mammalian/physiology; Eye/pathology; Female; Fibroblasts/cytology; Fibroblasts/metabolism; Genes, Lethal; Genes, Reporter; Humans; Intracellular Signaling Peptides and Proteins; Lens Diseases/genetics; Lens Diseases/metabolism; Mice; Mice, Transgenic; Nuclear Receptor Coactivators; Peptide Fragments/genetics; Peptide Fragments/metabolism; Phenotype; Pregnancy; Receptors, Cytoplasmic and Nuclear/metabolism; Receptors, Retinoic Acid/metabolism; Signal Transduction/physiology; Transcription Factors/genetics; Transcription Factors/metabolism |
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