نتایج جستجو برای: cdkl5

تعداد نتایج: 205  

Journal: :The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2013
Suvasini Sharma Asuri N Prasad

The epileptic encephalopathies of infancy are a group of disorders characterized by intractable seizures, persistent abnormality of cortical function documented on EEG, and consequently impaired neuro-developmental outcomes. The etiologies vary and include; structural brain malformations, acquired brain insults, and inborn errors of metabolism in the majority of the affected patients. In a prop...

2017
Ahmed Arafat Peng Jing Yuping Ma Miao Pu Gai Nan He Fang Chen Chen Yin Fei

Early Infantile Epileptic Encephalopathy (EIEE) presents shortly after birth with frequent, severe seizures and progressive disturbance of cerebral function. This study was to investigate a cohort of Chinese children with unexplained EIEE, infants with previous genetic diagnoses, causative brain malformations, or inborn errors of metabolism were excluded. We used targeted next-generation sequen...

2017
Dominik Reim Ute Distler Sonja Halbedl Chiara Verpelli Carlo Sala Juergen Bockmann Stefan Tenzer Tobias M. Boeckers Michael J. Schmeisser

Disruption of the human SHANK3 gene can cause several neuropsychiatric disease entities including Phelan-McDermid syndrome, autism spectrum disorder (ASD), and intellectual disability. Although, a wide array of neurobiological studies strongly supports a major role for SHANK3 in organizing the post-synaptic protein scaffold, the molecular processes at synapses of individuals harboring SHANK3 mu...

2015
Wendy Anne Gold John Christodoulou

Rett syndrome (RTT) is a rare, severe disorder of neuronal plasticity that predominantly affects girls. Girls with RTT usually appear asymptomatic in the first 6-18 months of life, but gradually develop severe motor, cognitive, and behavioral abnormalities that persist for life. A predominance of neuronal and synaptic dysfunction, with altered excitatory-inhibitory neuronal synaptic transmissio...

Journal: :Clinical genetics 2012
S Takahashi N Matsumoto A Okayama N Suzuki A Araki K Okajima H Tanaka A Miyamoto

Rett syndrome (RTT) is a severe neurodevelopmental disorder characterized by microcephaly, psychomotor regression, seizures and stereotypical hand movements. Recently, deletions and inactivating mutations in FOXG1, encoding a brain-specific transcription factor that is critical for forebrain development, have been found to be associated with the congenital variant of RTT. Here we report the cli...

2016
Robin T. Varghese Yanping Liang Ting Guan Christopher T. Franck Deborah F. Kelly Zhi Sheng

Cancer biomarkers with a strong predictive power for diagnosis/prognosis and a potential to be therapeutic targets have not yet been fully established. Here we employed a loss-of-function screen in glioblastoma (GBM), an infiltrative brain tumor with a dismal prognosis, and identified 20 survival kinase genes (SKGs). Survival analyses using The Cancer Genome Atlas (TCGA) datasets revealed that ...

Journal: :Molecular syndromology 2012
M H Willemsen J H M Rensen H M J van Schrojenstein-Lantman de Valk B C J Hamel T Kleefstra

BACKGROUND: Angelman- and Rett-like syndromes share a range of clinical characteristics, including intellectual disability (ID) with or without regression, epilepsy, infantile encephalopathy, postnatal microcephaly, features of autism spectrum disorder, and variable other neurological symptoms. The phenotypic spectrum generally has been well studied in children; however, evolution of the phenot...

Journal: :Cancer research 2011
Zhi Jiang Zang Choon Kiat Ong Ioana Cutcutache Willie Yu Shen Li Zhang Dachuan Huang Lian Dee Ler Karl Dykema Anna Gan Jiong Tao Siyu Lim Yujing Liu P Andrew Futreal Heike Grabsch Kyle A Furge Liang Kee Goh Steve Rozen Bin Tean Teh Patrick Tan

Genetic alterations in kinases have been linked to multiple human pathologies. To explore the landscape of kinase genetic variation in gastric cancer (GC), we used targeted, paired-end deep sequencing to analyze 532 protein and phosphoinositide kinases in 14 GC cell lines. We identified 10,604 single-nucleotide variants (SNV) in kinase exons including greater than 300 novel nonsynonymous SNVs. ...

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