نتایج جستجو برای: cdkl5

تعداد نتایج: 205  

Journal: :American journal of medical genetics. Part A 2006
Hayley L Archer Julie C Evans David S Millar Peter W Thompson Alison M Kerr Helen Leonard John Christodoulou David Ravine Lazarus Lazarou Lucy Grove Christopher Verity Sharon D Whatley Daniela T Pilz Julian R Sampson Angus J Clarke

A translocation that disrupted the netrin G1 gene (NTNG1) was recently reported in a patient with the early seizure variant of Rett syndrome (RTT). The netrin G1 protein (NTNG1) has an important role in the developing central nervous system, particularly in axonal guidance, signalling and NMDA receptor function and was a good candidate gene for RTT. We recruited 115 patients with RTT (females: ...

Journal: :Journal of medical genetics 2010
C Philippe D Amsallem C Francannet L Lambert A Saunier F Verneau P Jonveaux

BACKGROUND The FOXG1 gene has been recently implicated in the congenital form of Rett syndrome (RTT). It encodes the fork-head box protein G1, a winged-helix transcriptional repressor with expression restricted to testis and brain, where it is critical for forebrain development. So far, only two point mutations in FOXG1 have been reported in females affected by the congenital form of RTT. Aim T...

2009
Eric D. Marsh Jeffrey A. Golden

clinical overview The early epileptic encephalopathies are a group of conditions that all manifest with three major diagnostic criteria: medically refractory seizures, diffuse encephalopathy and a poor developmental outcome. These syndromes include early infantile epileptic encephalopathy (EEIE, also known as Otahara’s syndrome), severe myoclonic epilepsy of infancy (Dravet’s syndrome), infanti...

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