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Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity
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| Zeitschriftentitel: | The Journal of Neuroscience |
|---|---|
| Personen und Körperschaften: | , , , , , , , , , , , , |
| In: | The Journal of Neuroscience, 27, 2007, 35, S. 9439-9450 |
| Format: | E-Article |
| Sprache: | Englisch |
| veröffentlicht: |
Society for Neuroscience
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| Schlagwörter: |
| author_facet |
Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre |
|---|---|
| author |
Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre |
| spellingShingle |
Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre The Journal of Neuroscience Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity General Neuroscience |
| author_sort |
khelfaoui, malik |
| spelling |
Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre 0270-6474 1529-2401 Society for Neuroscience General Neuroscience http://dx.doi.org/10.1523/jneurosci.2029-07.2007 <jats:p>Loss of oligophrenin1 (OPHN1) function in human causes X-linked mental retardation associated with cerebellar hypoplasia and, in some cases, with lateral ventricle enlargement.<jats:italic>In vitro</jats:italic>studies showed that ophn1 regulates dendritic spine through the control of Rho GTPases, but its<jats:italic>in vivo</jats:italic>function remains unknown. We generated a mouse model of ophn1 deficiency and showed that it mimics the ventricles enlargement without affecting the cerebellum morphoanatomy. The ophn1 knock-out mice exhibit behavioral defects in spatial memory together with impairment in social behavior, lateralization, and hyperactivity. Long-term potentiation and mGluR-dependant long-term depression are normal in the CA1 hippocampal area of ophn1 mutant, whereas paired-pulse facilitation is reduced. This altered short-term plasticity that reflects changes in the release of neurotransmitters from the presynaptic processes is associated with normal synaptic density together with a reduction in mature dendritic spines. In culture, inactivation of ophn1 function increases the density and proportion of immature spines. Using a conditional model of loss of ophn1 function, we confirmed this immaturity defect and showed that ophn1 is required at all the stages of the development. These studies show that, depending of the context, ophn1 controls the maturation of dendritic spines either by maintaining the density of mature spines or by limiting the extension of new filopodia. Altogether, these observations indicate that cognitive impairment related to OPHN1 loss of function is associated with both presynaptic and postsynaptic alterations.</jats:p> Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity The Journal of Neuroscience |
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| title |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_unstemmed |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_full |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_fullStr |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_full_unstemmed |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_short |
Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_sort |
loss of x-linked mental retardation gene oligophrenin1 in mice impairs spatial memory and leads to ventricular enlargement and dendritic spine immaturity |
| topic |
General Neuroscience |
| url |
http://dx.doi.org/10.1523/jneurosci.2029-07.2007 |
| publishDate |
2007 |
| physical |
9439-9450 |
| description |
<jats:p>Loss of oligophrenin1 (OPHN1) function in human causes X-linked mental retardation associated with cerebellar hypoplasia and, in some cases, with lateral ventricle enlargement.<jats:italic>In vitro</jats:italic>studies showed that ophn1 regulates dendritic spine through the control of Rho GTPases, but its<jats:italic>in vivo</jats:italic>function remains unknown. We generated a mouse model of ophn1 deficiency and showed that it mimics the ventricles enlargement without affecting the cerebellum morphoanatomy. The ophn1 knock-out mice exhibit behavioral defects in spatial memory together with impairment in social behavior, lateralization, and hyperactivity. Long-term potentiation and mGluR-dependant long-term depression are normal in the CA1 hippocampal area of ophn1 mutant, whereas paired-pulse facilitation is reduced. This altered short-term plasticity that reflects changes in the release of neurotransmitters from the presynaptic processes is associated with normal synaptic density together with a reduction in mature dendritic spines. In culture, inactivation of ophn1 function increases the density and proportion of immature spines. Using a conditional model of loss of ophn1 function, we confirmed this immaturity defect and showed that ophn1 is required at all the stages of the development. These studies show that, depending of the context, ophn1 controls the maturation of dendritic spines either by maintaining the density of mature spines or by limiting the extension of new filopodia. Altogether, these observations indicate that cognitive impairment related to OPHN1 loss of function is associated with both presynaptic and postsynaptic alterations.</jats:p> |
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| author | Khelfaoui, Malik, Denis, Cécile, van Galen, Elly, de Bock, Frédéric, Schmitt, Alain, Houbron, Christophe, Morice, Elise, Giros, Bruno, Ramakers, Ger, Fagni, Laurent, Chelly, Jamel, Nosten-Bertrand, Marika, Billuart, Pierre |
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| description | <jats:p>Loss of oligophrenin1 (OPHN1) function in human causes X-linked mental retardation associated with cerebellar hypoplasia and, in some cases, with lateral ventricle enlargement.<jats:italic>In vitro</jats:italic>studies showed that ophn1 regulates dendritic spine through the control of Rho GTPases, but its<jats:italic>in vivo</jats:italic>function remains unknown. We generated a mouse model of ophn1 deficiency and showed that it mimics the ventricles enlargement without affecting the cerebellum morphoanatomy. The ophn1 knock-out mice exhibit behavioral defects in spatial memory together with impairment in social behavior, lateralization, and hyperactivity. Long-term potentiation and mGluR-dependant long-term depression are normal in the CA1 hippocampal area of ophn1 mutant, whereas paired-pulse facilitation is reduced. This altered short-term plasticity that reflects changes in the release of neurotransmitters from the presynaptic processes is associated with normal synaptic density together with a reduction in mature dendritic spines. In culture, inactivation of ophn1 function increases the density and proportion of immature spines. Using a conditional model of loss of ophn1 function, we confirmed this immaturity defect and showed that ophn1 is required at all the stages of the development. These studies show that, depending of the context, ophn1 controls the maturation of dendritic spines either by maintaining the density of mature spines or by limiting the extension of new filopodia. Altogether, these observations indicate that cognitive impairment related to OPHN1 loss of function is associated with both presynaptic and postsynaptic alterations.</jats:p> |
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| spelling | Khelfaoui, Malik Denis, Cécile van Galen, Elly de Bock, Frédéric Schmitt, Alain Houbron, Christophe Morice, Elise Giros, Bruno Ramakers, Ger Fagni, Laurent Chelly, Jamel Nosten-Bertrand, Marika Billuart, Pierre 0270-6474 1529-2401 Society for Neuroscience General Neuroscience http://dx.doi.org/10.1523/jneurosci.2029-07.2007 <jats:p>Loss of oligophrenin1 (OPHN1) function in human causes X-linked mental retardation associated with cerebellar hypoplasia and, in some cases, with lateral ventricle enlargement.<jats:italic>In vitro</jats:italic>studies showed that ophn1 regulates dendritic spine through the control of Rho GTPases, but its<jats:italic>in vivo</jats:italic>function remains unknown. We generated a mouse model of ophn1 deficiency and showed that it mimics the ventricles enlargement without affecting the cerebellum morphoanatomy. The ophn1 knock-out mice exhibit behavioral defects in spatial memory together with impairment in social behavior, lateralization, and hyperactivity. Long-term potentiation and mGluR-dependant long-term depression are normal in the CA1 hippocampal area of ophn1 mutant, whereas paired-pulse facilitation is reduced. This altered short-term plasticity that reflects changes in the release of neurotransmitters from the presynaptic processes is associated with normal synaptic density together with a reduction in mature dendritic spines. In culture, inactivation of ophn1 function increases the density and proportion of immature spines. Using a conditional model of loss of ophn1 function, we confirmed this immaturity defect and showed that ophn1 is required at all the stages of the development. These studies show that, depending of the context, ophn1 controls the maturation of dendritic spines either by maintaining the density of mature spines or by limiting the extension of new filopodia. Altogether, these observations indicate that cognitive impairment related to OPHN1 loss of function is associated with both presynaptic and postsynaptic alterations.</jats:p> Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity The Journal of Neuroscience |
| spellingShingle | Khelfaoui, Malik, Denis, Cécile, van Galen, Elly, de Bock, Frédéric, Schmitt, Alain, Houbron, Christophe, Morice, Elise, Giros, Bruno, Ramakers, Ger, Fagni, Laurent, Chelly, Jamel, Nosten-Bertrand, Marika, Billuart, Pierre, The Journal of Neuroscience, Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity, General Neuroscience |
| title | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_full | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_fullStr | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_full_unstemmed | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_short | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| title_sort | loss of x-linked mental retardation gene oligophrenin1 in mice impairs spatial memory and leads to ventricular enlargement and dendritic spine immaturity |
| title_unstemmed | Loss of X-Linked Mental Retardation Gene Oligophrenin1 in Mice Impairs Spatial Memory and Leads to Ventricular Enlargement and Dendritic Spine Immaturity |
| topic | General Neuroscience |
| url | http://dx.doi.org/10.1523/jneurosci.2029-07.2007 |