Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder
المؤلفون المشاركون
Fuchs, Claudia
Gennaccaro, Laura
Trazzi, Stefania
Bastianini, Stefano
Bettini, Simone
Martire, Viviana Lo
Ren, Elisa
Medici, Giorgio
Zoccoli, Giovanna
Rimondini, Roberto
Ciani, Elisabetta
المصدر
العدد
المجلد 2018، العدد 2018 (31 ديسمبر/كانون الأول 2018)، ص ص. 1-18، 18ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2018-05-27
دولة النشر
مصر
عدد الصفحات
18
التخصصات الرئيسية
الملخص EN
CDKL5 disorder is a severe neurodevelopmental disorder caused by mutations in the X-linked CDKL5 (cyclin-dependent kinase-like five) gene.
CDKL5 disorder primarily affects girls and is characterized by early-onset epileptic seizures, gross motor impairment, intellectual disability, and autistic features.
Although all CDKL5 female patients are heterozygous, the most valid disease-related model, the heterozygous female Cdkl5 knockout (Cdkl5 +/−) mouse, has been little characterized.
The lack of detailed behavioral profiling of this model remains a crucial gap that must be addressed in order to advance preclinical studies.
Here, we provide a behavioral and molecular characterization of heterozygous Cdkl5 +/− mice.
We found that Cdkl5 +/− mice reliably recapitulate several aspects of CDKL5 disorder, including autistic-like behaviors, defects in motor coordination and memory performance, and breathing abnormalities.
These defects are associated with neuroanatomical alterations, such as reduced dendritic arborization and spine density of hippocampal neurons.
Interestingly, Cdkl5 +/− mice show age-related alterations in protein kinase B (AKT) and extracellular signal-regulated kinase (ERK) signaling, two crucial signaling pathways involved in many neurodevelopmental processes.
In conclusion, our study provides a comprehensive overview of neurobehavioral phenotypes of heterozygous female Cdkl5 +/− mice and demonstrates that the heterozygous female might be a valuable animal model in preclinical studies on CDKL5 disorder.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Fuchs, Claudia& Gennaccaro, Laura& Trazzi, Stefania& Bastianini, Stefano& Bettini, Simone& Martire, Viviana Lo…[et al.]. 2018. Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder. Neural Plasticity،Vol. 2018, no. 2018, pp.1-18.
https://search.emarefa.net/detail/BIM-1210558
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Fuchs, Claudia…[et al.]. Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder. Neural Plasticity No. 2018 (2018), pp.1-18.
https://search.emarefa.net/detail/BIM-1210558
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Fuchs, Claudia& Gennaccaro, Laura& Trazzi, Stefania& Bastianini, Stefano& Bettini, Simone& Martire, Viviana Lo…[et al.]. Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder. Neural Plasticity. 2018. Vol. 2018, no. 2018, pp.1-18.
https://search.emarefa.net/detail/BIM-1210558
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1210558
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر