نتایج جستجو برای: cdkl5

تعداد نتایج: 205  

Journal: :European Journal of Human Genetics 2011

Journal: :Journal of Sleep Research 2017

Journal: :International journal of molecular sciences 2016
Valentina Imperatore Maria Antonietta Mencarelli Chiara Fallerini Laura Bianciardi Francesca Ariani Simone Furini Alessandra Renieri Francesca Mari Elisa Frullanti

We highlight the importance of exome sequencing in solving a clinical case of a child who died at 14 months after a series of respiratory crises. He was the half-brother of a girl diagnosed at 7 years with the early-onset seizure variant of Rett syndrome due to CDKL5 mutation. We performed a test for CDKL5 in the boy, which came back negative. Driven by the mother's compelling need for a diagno...

2014
Sylvie Bourthoumieu Cécile Laroche Aziza Lebbar Jean-Michel Dupont Catherine Yardin

We describe two cases where clinical diagnoses were carried out (Rett syndrome-like and Nicolaides-Baraitser syndrome) with no identified mutation respectively in the CDKL5 and the SMARCA2 genes. Conversely a chromosomal microdeletion with contiguous deletion of a part of these two genes was found by arrayCGH in each corresponding case. The aim of this report is then to highlight the possible i...

2015
Anna Ka-Yee Kwong Alvin Chi-Chung Ho Cheuk-Wing Fung Virginia Chun-Nei Wong

Epileptic Encephalopathy (EE) is a heterogeneous condition in which cognitive, sensory and/or motor functions deteriorate as a consequence of epileptic activity, which consists of frequent seizures and/or major interictal paroxysmal activity. There are various causes of EE and they may occur at any age in early childhood. Genetic mutations have been identified to contribute to an increasing num...

Journal: :International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience 2015
José Pedro Vieira Fátima Lopes Anabela Silva-Fernandes Maria Vânia Sousa Sofia Moura Susana Sousa Bruno M Costa Mafalda Barbosa Bauke Ylstra Teresa Temudo Teresa Lourenço Patrícia Maciel

Rett syndrome is a neurodevelopmental disorder caused by mutations in the MECP2 gene. We investigated the genetic basis of disease in a female patient with a Rett-like clinical. Karyotype analysis revealed a pericentric inversion in the X chromosome -46,X,inv(X)(p22.1q28), with breakpoints in the cytobands where the MECP2 and CDKL5 genes are located. FISH analysis revealed that the MECP2 gene i...

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