[article]
| Titre : |
Firing activity of locus coeruleus noradrenergic neurons decreases in necdin-deficient mice, an animal model of Prader-Willi syndrome |
| Type de document : |
texte imprimé |
| Auteurs : |
Rui-Ni WU, Auteur ; Wei-Chen HUNG, Auteur ; Ching-Tsuey CHEN, Auteur ; Li-Ping TSAI, Auteur ; Wen-Sung LAI, Auteur ; Ming-Yuan MIN, Auteur ; Shi-Bing WONG, Auteur |
| Langues : |
Anglais (eng) |
| Mots-clés : |
Adrenergic Neurons/metabolism Animals Animals, Newborn Disease Models, Animal Female Gene Expression Regulation, Developmental Locus Coeruleus/metabolism Mice Nerve Tissue Proteins Nuclear Proteins Prader-Willi Syndrome/metabolism A-type potassium current Hypercapnia Hypotonia Locus coeruleus Necdin Prader–Willi syndrome |
| Index. décimale : |
PER Périodiques |
| Résumé : |
BACKGROUND: Prader-Willi syndrome (PWS) is a neurodevelopmental disorder characterized by multiple respiratory, cognitive, endocrine, and behavioral symptoms, such as central apnea, intellectual disabilities, exaggerated stress responses, and temper tantrums. The locus coeruleus noradrenergic system (LC-NE) modulates a diverse range of behaviors, including arousal, learning, pain modulation, and stress-induced negative affective states, which are possibly correlated with the pathogenesis of PWS phenotypes. Therefore, we evaluated the LC-NE neuronal activity of necdin-deficient mice, an animal model of PWS. METHODS: Heterozygous necdin-deficient mice (B6.Cg-Ndn(tm1ky)) were bred from wild-type (WT) females to generate WT (+m/+p) and heterozygotes (+m/-p) animals, which were examined of LC-NE neuronal activity, developmental reflexes, and plethysmography. RESULTS: On slice electrophysiology, LC-NE neurons of Ndn(tm1ky) mice with necdin deficiency showed significantly decreased spontaneous activities and impaired excitability, which was mediated by enhanced A-type voltage-dependent potassium currents. Ndn(tm1ky) mice also exhibited the neonatal phenotypes of PWS, such as hypotonia and blunt respiratory responses to hypercapnia. CONCLUSIONS: LC-NE neuronal firing activity decreased in necdin-deficient mice, suggesting that LC, the primary source of norepinephrine in the central nervous system, is possibly involved in PWS pathogenesis. |
| En ligne : |
https://dx.doi.org/10.1186/s11689-020-09323-4 |
| Permalink : |
https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=573 |
in Journal of Neurodevelopmental Disorders > 12 (2020)
[article] Firing activity of locus coeruleus noradrenergic neurons decreases in necdin-deficient mice, an animal model of Prader-Willi syndrome [texte imprimé] / Rui-Ni WU, Auteur ; Wei-Chen HUNG, Auteur ; Ching-Tsuey CHEN, Auteur ; Li-Ping TSAI, Auteur ; Wen-Sung LAI, Auteur ; Ming-Yuan MIN, Auteur ; Shi-Bing WONG, Auteur. Langues : Anglais ( eng) in Journal of Neurodevelopmental Disorders > 12 (2020)
| Mots-clés : |
Adrenergic Neurons/metabolism Animals Animals, Newborn Disease Models, Animal Female Gene Expression Regulation, Developmental Locus Coeruleus/metabolism Mice Nerve Tissue Proteins Nuclear Proteins Prader-Willi Syndrome/metabolism A-type potassium current Hypercapnia Hypotonia Locus coeruleus Necdin Prader–Willi syndrome |
| Index. décimale : |
PER Périodiques |
| Résumé : |
BACKGROUND: Prader-Willi syndrome (PWS) is a neurodevelopmental disorder characterized by multiple respiratory, cognitive, endocrine, and behavioral symptoms, such as central apnea, intellectual disabilities, exaggerated stress responses, and temper tantrums. The locus coeruleus noradrenergic system (LC-NE) modulates a diverse range of behaviors, including arousal, learning, pain modulation, and stress-induced negative affective states, which are possibly correlated with the pathogenesis of PWS phenotypes. Therefore, we evaluated the LC-NE neuronal activity of necdin-deficient mice, an animal model of PWS. METHODS: Heterozygous necdin-deficient mice (B6.Cg-Ndn(tm1ky)) were bred from wild-type (WT) females to generate WT (+m/+p) and heterozygotes (+m/-p) animals, which were examined of LC-NE neuronal activity, developmental reflexes, and plethysmography. RESULTS: On slice electrophysiology, LC-NE neurons of Ndn(tm1ky) mice with necdin deficiency showed significantly decreased spontaneous activities and impaired excitability, which was mediated by enhanced A-type voltage-dependent potassium currents. Ndn(tm1ky) mice also exhibited the neonatal phenotypes of PWS, such as hypotonia and blunt respiratory responses to hypercapnia. CONCLUSIONS: LC-NE neuronal firing activity decreased in necdin-deficient mice, suggesting that LC, the primary source of norepinephrine in the central nervous system, is possibly involved in PWS pathogenesis. |
| En ligne : |
https://dx.doi.org/10.1186/s11689-020-09323-4 |
| Permalink : |
https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=573 |
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Firing activity of locus coeruleus noradrenergic neurons decreases in necdin-deficient mice, an animal model of Prader-Willi syndrome in Journal of Neurodevelopmental Disorders, 12 (2020)
