Linked Life Data

18093976

Source:http://linkedlifedata.com/resource/pubmed/id/18093976

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2008-2-25
pubmed:abstractText
Spinal muscular atrophy (SMA) is caused by reduced levels of the survival of motor neuron (SMN) protein. Although the SMN complex is essential for assembly of spliceosomal U small nuclear RNPs, it is still not understood why reduced levels of the SMN protein specifically cause motor neuron degeneration. SMN was recently proposed to have specific functions in mRNA transport and translation regulation in neuronal processes. The defective protein in Fragile X mental retardation syndrome (FMRP) also plays a role in transport of mRNPs and in their translation. Therefore, we examined possible relationships of SMN with FMRP. We observed granules containing both transiently expressed red fluorescent protein(RFP)-tagged SMN and green fluorescent protein(GFP)-tagged FMRP in cell bodies and processes of rat primary neurons of hypothalamus in culture. By immunoprecipitation experiments, we detected an association of FMRP with the SMN complex in human neuroblastoma SH-SY5Y cells and in murine motor neuron MN-1 cells. Then, by in vitro experiments, we demonstrated that the SMN protein is essential for this association. We showed that the COOH-terminal region of FMRP, as well as the conserved YG box and the region encoded by exon 7 of SMN, are required for the interaction. Our findings suggest a link between the SMN complex and FMRP in neuronal cells.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0021-9258
pubmed:author
pubmed:issnType
Print
pubmed:day
29
pubmed:volume
283
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
5598-610
pubmed:dateRevised
2008-11-21
pubmed:meshHeading
pubmed-meshheading:18093976-Animals, pubmed-meshheading:18093976-Cell Line, Tumor, pubmed-meshheading:18093976-Cyclic AMP Response Element-Binding Protein, pubmed-meshheading:18093976-Exons, pubmed-meshheading:18093976-Fragile X Mental Retardation Protein, pubmed-meshheading:18093976-Fragile X Syndrome, pubmed-meshheading:18093976-Humans, pubmed-meshheading:18093976-Hypothalamus, pubmed-meshheading:18093976-Mice, pubmed-meshheading:18093976-Motor Neurons, pubmed-meshheading:18093976-Muscular Atrophy, Spinal, pubmed-meshheading:18093976-Nerve Tissue Proteins, pubmed-meshheading:18093976-Protein Structure, Tertiary, pubmed-meshheading:18093976-RNA-Binding Proteins, pubmed-meshheading:18093976-Recombinant Fusion Proteins, pubmed-meshheading:18093976-SMN Complex Proteins, pubmed-meshheading:18093976-Survival of Motor Neuron 1 Protein
pubmed:year
2008
pubmed:articleTitle
In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein.
pubmed:affiliation
Laboratoire de Maturation des ARN et Enzymologie Moléculaire, UMR 7567 CNRS-UHP Nancy I, Faculté des Sciences, BP 239, 54506 Vandoeuvre-les-Nancy Cedex.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't