نتایج جستجو برای: angelman syndrome

تعداد نتایج: 621986  

Journal: :JAMA pediatrics 2016
Francesco M Galassi Emanuele Armocida Frank J Rühli

2016
Tímea Margit Szabó Anikó Ujfalusi Beáta Bessenyei Gabriella P. Szabó Katalin Szakszon István Balogh Éva Oláh

15q11-13 chromosome region contains five breakpoints (BP1-BP5). Chromosomal rearrangements are common in this region. The microdeletion of BP1-BP2 region represents the 15q11.2 microdeletion syndrome associating with variable phenotype. We investigated a ten years old boy with hypotony. His motoric functions, speech and intellectual development were delayed. He suffered from epilepsy and showed...

Journal: :European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2009
Bernard Dan Karine Pelc Catherine Christophe

Scientific knowledge relies on sound evidence. In many domains the latter is still erratic, so that the former is provisionally replaced by inference based on scant data provided unsatisfactorily answered questions remain critically open for scrutiny. Neuroimaging and pathology of Angelman syndrome provide an illustration of this process. Although this condition is primarily characterised by va...

Journal: :Journal of neurophysiology 2014
Jada J Hallengren Ryan J Vaden

Angelman syndrome is a neurodevelopmental disorder characterized by intellectual disabilities, ataxia, and unusually happy affect. The hippocampal pyramidal cells of Angelman syndrome model mice have altered intrinsic membrane properties, which Kaphzan et al. (Cell Rep 4: 405-412, 2013) demonstrate can be corrected by genetic reduction of the α1-subunit of the sodium-potassium ATPase. Intriguin...

Journal: :Seizure 2008
Karine Pelc Stewart G. Boyd Guy Cheron Bernard Dan

Angelman syndrome is a neurogenetic disorder caused by lack of UBE3A gene expression from the maternally inherited chromosome 15 due to various 15q11-q13 abnormalities. In addition to severe developmental delay, virtual absence of speech, motor impairment, a behavioural phenotype that includes happy demeanor, and distinctive rhythmic electroencephalographic features, over 90% of patients have e...

Journal: :European Journal of Human Genetics 2009

Journal: :Revista Española de Enfermedades Digestivas 2010

Journal: :Brain & development 2005
Tomohiro Kumada Masatoshi Ito Tomoko Miyajima Tatsuya Fujii Takehiko Okuno Toshin Go Haruo Hattori Mieko Yoshioka Kenichiro Kobayashi Osamu Kanazawa Jun Tohyama Noriyuki Akasaka Takanori Kamimura Mutsuo Sasagawa Hideki Amagane Kozo Mutoh Yuriko Yamori Toyoko Kanda Naoko Yoshida Haruyo Hirota Rieko Tanaka Yasushi Hamada

While there is an abundance of literature describing the association of chromosome aberrations with epilepsy, only a few refer to the detailed features of epilepsy. It is important to investigate the associations between specific chromosome abnormalities and features of epilepsy to identify genes involved in epilepsy and treat them more effectively. We investigated the correlation between speci...

Journal: :Indian Journal of Anaesthesia 2017

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