The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses. Export

@article{citeulike:552159, abstract = {The Fragile X syndrome, which results from the absence of functional FMRP protein, is the most common heritable form of mental retardation. Here, we show that FMRP acts as a translational repressor of specific mRNAs at synapses. Interestingly, FMRP associates not only with these target mRNAs, but also with the dendritic, non-translatable RNA BC1. Blocking of BC1 inhibits the interaction of FMRP with its target mRNAs. Furthermore, BC1 binds directly to FMRP and can also associate, in the absence of any protein, with the mRNAs regulated by FMRP. This suggests a mechanism where BC1 could determine the specificity of FMRP function by linking the regulated mRNAs and FMRP. Thus, when FMRP is not present, loss of translational repression of specific mRNAs at synapses could result in synaptic dysfunction phenotype of Fragile X patients.}, address = {Dipartimento di Biologia, Universit\`{a} di Roma, Tor Vergata, Via della Ricerca Scientifica, 00133 Roma, Italy.}, author = {Zalfa, F. and Giorgi, M. and Primerano, B. and Moro, A. and Di Penta, A. and Reis, S. and Oostra, B. and Bagni, C.}, citeulike-article-id = {552159}, citeulike-linkout-0 = {http://view.ncbi.nlm.nih.gov/pubmed/12581522}, citeulike-linkout-1 = {http://www.hubmed.org/display.cgi?uids=12581522}, day = {7}, issn = {0092-8674}, journal = {Cell}, keywords = {bc1, disorder, fmrp, ncrna, neural}, month = {February}, number = {3}, pages = {317--327}, posted-at = {2006-03-15 02:38:23}, priority = {2}, title = {The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses.}, url = {http://view.ncbi.nlm.nih.gov/pubmed/12581522}, volume = {112}, year = {2003} }

TY - JOUR ID - citeulike:552159 L3 - citeulike-article-id:552159 N2 - The Fragile X syndrome, which results from the absence of functional FMRP protein, is the most common heritable form of mental retardation. Here, we show that FMRP acts as a translational repressor of specific mRNAs at synapses. Interestingly, FMRP associates not only with these target mRNAs, but also with the dendritic, non-translatable RNA BC1. Blocking of BC1 inhibits the interaction of FMRP with its target mRNAs. Furthermore, BC1 binds directly to FMRP and can also associate, in the absence of any protein, with the mRNAs regulated by FMRP. This suggests a mechanism where BC1 could determine the specificity of FMRP function by linking the regulated mRNAs and FMRP. Thus, when FMRP is not present, loss of translational repression of specific mRNAs at synapses could result in synaptic dysfunction phenotype of Fragile X patients. IS - 3 JF - Cell SN - 0092-8674 AD - Dipartimento di Biologia, Università di Roma, Tor Vergata, Via della Ricerca Scientifica, 00133 Roma, Italy. EP - 327 TI - The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses. VL - 112 SP - 317 KW - bc1 KW - disorder KW - fmrp KW - ncrna KW - neural AU - Zalfa, F AU - Giorgi, M AU - Primerano, B AU - Moro, A AU - Di Penta, A AU - Reis, S AU - Oostra, B AU - Bagni, C PY - 2003/02/07/ UR - http://view.ncbi.nlm.nih.gov/pubmed/12581522 ER -