title: A heterogeneous set of FMR1 proteins is widely distributed in mouse tissues and is modulated in cell culture.
abstract: The fragile X syndrome is an X-linked inherited disease and is the result of transcriptional inactivation of the FMR1 gene and the absence of its encoded FMR protein (FMRP). Using a specific monoclonal 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 mRNA, although at different levels, and contain several additional shorter transcripts, particularly in muscular tissues. We also report that expression of the FMR1 gene is modulated in proliferating and quiescent primary mouse kidney cell cultures with an inverse relationship between levels of FMR1 mRNA and of its encoded proteins. This suggests that FMRPs are highly stable in quiescent cells and that FMR1 expression is likely post-transcriptionally controlled. Our results document the widespread expression of the FMR1 gene, and suggest that it is controlled by different mechanisms implicated in cell growth and differentiation.
authors.affiliation: Unité de recherche en génétique humaine et moléculaire, Hôpital Saint François d'Assise, Québec, Canada.
authors.forename: E W
authors.initials: EW
authors.lastname: Khandjian
authors.forename: A
authors.initials: A
authors.lastname: Fortin
authors.forename: A
authors.initials: A
authors.lastname: Thibodeau
authors.forename: S
authors.initials: S
authors.lastname: Tremblay
authors.forename: F
authors.initials: F
authors.lastname: Côté
authors.forename: D
authors.initials: D
authors.lastname: Devys
authors.forename: J L
authors.initials: JL
authors.lastname: Mandel
authors.forename: F
authors.initials: F
authors.lastname: Rousseau
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal; D002478:Cells, Cultured; D005260:Female; D051860:Fragile X Messenger Ribonucleoprotein 1; D005600:Fragile X Syndrome / Q000235:genetics; D015870:Gene Expression; D006801:Humans; D007668:Kidney / Q000378:metabolism; D008297:Male; D051379:Mice; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000276:immunology / Q000378:metabolism*; D011499:Protein Processing, Post-Translational; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D016601:RNA-Binding Proteins; D014018:Tissue Distribution
chemical_list: D000911:Antibodies, Monoclonal; D051860:Fragile X Messenger Ribonucleoprotein 1; D009419:Nerve Tissue Proteins; D012333:RNA, Messenger; D016601:RNA-Binding Proteins; C497683:FMR1 protein, human; C497684:Fmr1 protein, mouse