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- Title
A heterogeneous set of FMR1 proteins is widely distributed in mouse tissues and is modulated in cell culture.
- Authors
Khandjian, Edouard W.; Anny, Fortin; Thibodeau, Alain; Tremblay, Sandra; Côt´, France; Devys, Didier; Mandel, Jean-Louis; Rousseau, François
- Abstract
The fragile X syndrome is an X-linked inherited disease and is the result of transcriptional inactivation of the gene and the absence of its encoded FMR protein (FMRP). Using a specific monocional antibody directed against human FMRP, we have studied the steady-state levels of its murine homolog in several tissues and organs of adult and young mice. In immunoblot analyses, the antibody recognizes a heterogeneous subset of proteins with apparent molecular weights ranging from 80 to 70 kDa. These proteins are detected in all the 27 tissues tested; however, the relative proportion of each polypeptide recognized varies between tissues, and a significantly higher expression Is observed in young animals. Northern blot analysis of RNA extracted from selected tissues from adult mouse shows that these tissues express the major 4.8 kb mANA, although at different levels, and contain several additional shorter transcripts, particularly In muscular tissues. We also report that expression of the gene is modulated in proliferating and quiescent primary mouse kidney cell cultures with an inverse relationship between levels of mRNAJ and of its encoded proteins. This suggests that FMRPS are highly stable in quiescent cells and that expression is likely post transcriptionally controlled. Our results document the widespread expression of the gene, and suggest that it is controlled by different mechanisms implicated in cell growth and differentiation.
- Publication
Human Molecular Genetics, 1995, Vol 4, Issue 5, p783
- ISSN
0964-6906
- Publication type
Article