Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression

Research output: Contribution to journal › Journal article › Research › peer-review

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Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression. / Mouton-Liger, Francois; Sahun, Ignasi; Collin, Thibault; Pereira, Patricia Lopes; Masini, Debora; Thomas, Sophie; Paly, Evelyne; Luilier, Sabrina; Meme, Sandra; Jouhault, Quentin; Bennai, Soumia; Beloeil, Jean-Claude; Bizot, Jean-Charles; Herault, Yann; Dierssen, Mara; Creau, Nicole.

In: Neurobiology of Disease, Vol. 63, 03.2014, p. 92-106.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Mouton-Liger, F, Sahun, I, Collin, T, Pereira, PL, Masini, D, Thomas, S, Paly, E, Luilier, S, Meme, S, Jouhault, Q, Bennai, S, Beloeil, J-C, Bizot, J-C, Herault, Y, Dierssen, M & Creau, N 2014, 'Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression', Neurobiology of Disease, vol. 63, pp. 92-106. https://doi.org/10.1016/j.nbd.2013.11.016

APA

Mouton-Liger, F., Sahun, I., Collin, T., Pereira, P. L., Masini, D., Thomas, S., Paly, E., Luilier, S., Meme, S., Jouhault, Q., Bennai, S., Beloeil, J-C., Bizot, J-C., Herault, Y., Dierssen, M., & Creau, N. (2014). Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression. Neurobiology of Disease, 63, 92-106. https://doi.org/10.1016/j.nbd.2013.11.016

Vancouver

Mouton-Liger F, Sahun I, Collin T, Pereira PL, Masini D, Thomas S et al. Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression. Neurobiology of Disease. 2014 Mar;63:92-106. https://doi.org/10.1016/j.nbd.2013.11.016

Author

Mouton-Liger, Francois ; Sahun, Ignasi ; Collin, Thibault ; Pereira, Patricia Lopes ; Masini, Debora ; Thomas, Sophie ; Paly, Evelyne ; Luilier, Sabrina ; Meme, Sandra ; Jouhault, Quentin ; Bennai, Soumia ; Beloeil, Jean-Claude ; Bizot, Jean-Charles ; Herault, Yann ; Dierssen, Mara ; Creau, Nicole. / Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression. In: Neurobiology of Disease. 2014 ; Vol. 63. pp. 92-106.

Bibtex

@article{e77c0751921b444787cf2634c5ec3329,
title = "Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression",
abstract = "PCP4/PEP19 is a modulator of Ca2+ -CaM signaling. In the brain, it is expressed in a very specific pattern in postmitotic neurons. In particular, Pcp4 is highly expressed in the Purkinje cell, the sole output neuron of the cerebellum. PCP4, located on human chromosome 21, is present in three copies in individuals with Down syndrome (DS). In a previous study using a transgenic mouse model (TgPCP4) to evaluate the consequences of 3 copies of this gene, we found that PCP4 overexpression induces precocious neuronal differentiation during mouse embryogenesis. Here, we report combined analyses of the cerebellum at postnatal stages (P14 and adult) in which we identified age related molecular, electrophysiological, and behavioral alterations in the TgPCP4 mouse. While Pcp4 overexpression at P14 induces an earlier neuronal maturation, at adult stage it induces increase in cerebellar CaMK2alpha and in cerebellar LTD, as well as learning impairments. We therefore propose that PCP4 contributes significantly to the development of Down syndrome phenotypes through molecular and functional changes. (c) 2013 Elsevier Inc. All rights reserved.",
keywords = "Pcp4, Transgenic mouse, Down syndrome, Cerebellum, Learning, DEPENDENT PROTEIN-KINASE, D-ASPARTATE RECEPTORS, MOUSE MODEL, GENE-EXPRESSION, SYNAPTIC PLASTICITY, PURKINJE-CELLS, MESSENGER-RNA, ALPHA-CAMKII, NEURONAL DIFFERENTIATION, ROR-ALPHA",
author = "Francois Mouton-Liger and Ignasi Sahun and Thibault Collin and Pereira, {Patricia Lopes} and Debora Masini and Sophie Thomas and Evelyne Paly and Sabrina Luilier and Sandra Meme and Quentin Jouhault and Soumia Bennai and Jean-Claude Beloeil and Jean-Charles Bizot and Yann Herault and Mara Dierssen and Nicole Creau",
year = "2014",
month = mar,
doi = "10.1016/j.nbd.2013.11.016",
language = "English",
volume = "63",
pages = "92--106",
journal = "Neurobiology of Disease",
issn = "0969-9961",
publisher = "Academic Press",

}

RIS

TY - JOUR

T1 - Developmental molecular and functional cerebellar alterations induced by PCP4/PEP19 overexpression

AU - Mouton-Liger, Francois

AU - Sahun, Ignasi

AU - Collin, Thibault

AU - Pereira, Patricia Lopes

AU - Masini, Debora

AU - Thomas, Sophie

AU - Paly, Evelyne

AU - Luilier, Sabrina

AU - Meme, Sandra

AU - Jouhault, Quentin

AU - Bennai, Soumia

AU - Beloeil, Jean-Claude

AU - Bizot, Jean-Charles

AU - Herault, Yann

AU - Dierssen, Mara

AU - Creau, Nicole

PY - 2014/3

Y1 - 2014/3

N2 - PCP4/PEP19 is a modulator of Ca2+ -CaM signaling. In the brain, it is expressed in a very specific pattern in postmitotic neurons. In particular, Pcp4 is highly expressed in the Purkinje cell, the sole output neuron of the cerebellum. PCP4, located on human chromosome 21, is present in three copies in individuals with Down syndrome (DS). In a previous study using a transgenic mouse model (TgPCP4) to evaluate the consequences of 3 copies of this gene, we found that PCP4 overexpression induces precocious neuronal differentiation during mouse embryogenesis. Here, we report combined analyses of the cerebellum at postnatal stages (P14 and adult) in which we identified age related molecular, electrophysiological, and behavioral alterations in the TgPCP4 mouse. While Pcp4 overexpression at P14 induces an earlier neuronal maturation, at adult stage it induces increase in cerebellar CaMK2alpha and in cerebellar LTD, as well as learning impairments. We therefore propose that PCP4 contributes significantly to the development of Down syndrome phenotypes through molecular and functional changes. (c) 2013 Elsevier Inc. All rights reserved.

AB - PCP4/PEP19 is a modulator of Ca2+ -CaM signaling. In the brain, it is expressed in a very specific pattern in postmitotic neurons. In particular, Pcp4 is highly expressed in the Purkinje cell, the sole output neuron of the cerebellum. PCP4, located on human chromosome 21, is present in three copies in individuals with Down syndrome (DS). In a previous study using a transgenic mouse model (TgPCP4) to evaluate the consequences of 3 copies of this gene, we found that PCP4 overexpression induces precocious neuronal differentiation during mouse embryogenesis. Here, we report combined analyses of the cerebellum at postnatal stages (P14 and adult) in which we identified age related molecular, electrophysiological, and behavioral alterations in the TgPCP4 mouse. While Pcp4 overexpression at P14 induces an earlier neuronal maturation, at adult stage it induces increase in cerebellar CaMK2alpha and in cerebellar LTD, as well as learning impairments. We therefore propose that PCP4 contributes significantly to the development of Down syndrome phenotypes through molecular and functional changes. (c) 2013 Elsevier Inc. All rights reserved.

KW - Pcp4

KW - Transgenic mouse

KW - Down syndrome

KW - Cerebellum

KW - Learning

KW - DEPENDENT PROTEIN-KINASE

KW - D-ASPARTATE RECEPTORS

KW - MOUSE MODEL

KW - GENE-EXPRESSION

KW - SYNAPTIC PLASTICITY

KW - PURKINJE-CELLS

KW - MESSENGER-RNA

KW - ALPHA-CAMKII

KW - NEURONAL DIFFERENTIATION

KW - ROR-ALPHA

U2 - 10.1016/j.nbd.2013.11.016

DO - 10.1016/j.nbd.2013.11.016

M3 - Journal article

VL - 63

SP - 92

EP - 106

JO - Neurobiology of Disease

JF - Neurobiology of Disease

SN - 0969-9961

ER -

ID: 248194781