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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
2
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pubmed:dateCreated |
2009-1-22
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pubmed:abstractText |
The lack of fragile X mental retardation protein (FMRP) causes fragile X syndrome, a common form of inherited mental retardation. Our previous studies revealed alterations in the differentiation of FMRP-deficient neural progenitors. Here, we show abnormalities in neurogenesis in the mouse and human embryonic FMRP-deficient brain as well as after in utero transfection of I304N mutated FMRP, which acts in a dominant negative manner in the wild-type mouse brain. Progenitors accumulated abnormally in the subventricular zone of the embryonic Fmr1-knockout (Fmr1-KO) mouse neocortex. An increased density of cells expressing sequentially an intermediate progenitor marker, T-box transcription factor (Tbr2), and a postmitotic neuron marker, T-brain 1 (Tbr1), indicated that the differentiation to glutamatergic cell lineages was particularly disturbed. These abnormalities were associated with an increased density of pyramidal cells of the layer V in the early postnatal neocortex suggesting a role for FMRP in the regulation of the differentiation of neocortical glutamatergic neurons.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Eomes protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Excitatory Amino Acid Transporter 1,
http://linkedlifedata.com/resource/pubmed/chemical/FMR1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Fabp7 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Fatty Acid-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Fmr1 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Fragile X Mental Retardation Protein,
http://linkedlifedata.com/resource/pubmed/chemical/Glutamic Acid,
http://linkedlifedata.com/resource/pubmed/chemical/Nerve Tissue Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Slc1a3 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/T-Box Domain Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Tbr1 protein, mouse
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pubmed:status |
MEDLINE
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pubmed:month |
Feb
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pubmed:issn |
1095-953X
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pubmed:author |
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pubmed:issnType |
Electronic
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pubmed:volume |
33
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
250-9
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pubmed:meshHeading |
pubmed-meshheading:19056494-Animals,
pubmed-meshheading:19056494-Animals, Newborn,
pubmed-meshheading:19056494-Cell Differentiation,
pubmed-meshheading:19056494-Cell Lineage,
pubmed-meshheading:19056494-DNA-Binding Proteins,
pubmed-meshheading:19056494-Disease Models, Animal,
pubmed-meshheading:19056494-Excitatory Amino Acid Transporter 1,
pubmed-meshheading:19056494-Fatty Acid-Binding Proteins,
pubmed-meshheading:19056494-Fragile X Mental Retardation Protein,
pubmed-meshheading:19056494-Fragile X Syndrome,
pubmed-meshheading:19056494-Glutamic Acid,
pubmed-meshheading:19056494-Mesenchymal Stem Cells,
pubmed-meshheading:19056494-Mice,
pubmed-meshheading:19056494-Mice, Knockout,
pubmed-meshheading:19056494-Mutation,
pubmed-meshheading:19056494-Neocortex,
pubmed-meshheading:19056494-Nerve Tissue Proteins,
pubmed-meshheading:19056494-Neurogenesis,
pubmed-meshheading:19056494-Neurons,
pubmed-meshheading:19056494-Pyramidal Cells,
pubmed-meshheading:19056494-Stem Cells,
pubmed-meshheading:19056494-T-Box Domain Proteins
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pubmed:year |
2009
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pubmed:articleTitle |
Aberrant differentiation of glutamatergic cells in neocortex of mouse model for fragile X syndrome.
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pubmed:affiliation |
Neuroscience Center, University of Helsinki, P.O. Box 56, 00014 Helsinki, Finland.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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