نتایج جستجو برای: lissencephaly

تعداد نتایج: 686  

Journal: :The EMBO journal 2008
Masami Yamada Shiori Toba Yuko Yoshida Koji Haratani Daisuke Mori Yoshihisa Yano Yuko Mimori-Kiyosue Takeshi Nakamura Kyoko Itoh Shinji Fushiki Mitsutoshi Setou Anthony Wynshaw-Boris Takayuki Torisawa Yoko Y Toyoshima Shinji Hirotsune

LIS1 was first identified as a gene mutated in human classical lissencephaly sequence. LIS1 is required for dynein activity, but the underlying mechanism is poorly understood. Here, we demonstrate that LIS1 suppresses the motility of cytoplasmic dynein on microtubules (MTs), whereas NDEL1 releases the blocking effect of LIS1 on cytoplasmic dynein. We demonstrate that LIS1, cytoplasmic dynein an...

2005
Jerrell C. Cassady Lawrence L. Smith

This study examined the effects of the Waterford Early Reading Program on reading achievement gains across the first-grade year. The results of this longitudinal analysis demonstrated meaningfully greater gains on reading achievement for those with access to the Integrated learning system (ILS) (g 1⁄4 .10). Furthermore, the positive effects associated with access to the ILS were greatest for st...

Journal: :American Journal of Medical Genetics Part A 2016

Journal: :Clinical dysmorphology 2005
Omar Abu-Sa'da Maha Barbar Naffaa Al-Harbi Doris Taha

ARC syndrome, the association of arthrogryposis, renal tubular dysfunction and cholestasis, is a rare genetic disorder. We report two Saudi infants from two different families with ARC syndrome. Magnetic resonance imaging of the brain of one of the infants showed lissencephaly, a previously unreported finding in this syndrome. We also review 39 ARC cases reported in the literature using the Med...

Journal: :AJNR. American journal of neuroradiology 2012
E K Bravo M L White A H Olney J L McAllister Y D Zhang

We report the unique CNS findings in a patient with a proximal chromosome 14q interstitial deletion. Conventional MR imaging allowed the clear delineation of agenesis of the corpus callosum, SOD, and diffuse lissencephaly. DTI tractography played a significant role in the evaluation of the proximal 14q deletion-associated abnormalities, delineating the extent of the dysmorphic connections of th...

Journal: :Seizure 2007
Thorsten Gerstner Marc-Oliver Bauer Elke Longin Nellie Bell Stephan A. Koenig

Valproic acid (VPA) is considered to be a drug of first choice for the therapy of generalized and focal epilepsies, including special epileptic syndromes like the WEST-syndrome. The drug is usually well tolerated; rare serious complications may occur in some patients, including haemorrhagic pancreatitis, coagulapathies, bone marrow suppression, VPA-induced hepatotoxicity and encephalopathy. We ...

Journal: :Archives of disease in childhood 1991
N Sharief J Craze D Summers L Butler C B Wood

A girl presented at 6 weeks of age with failure to thrive and arching of the back. She had various dysmorphic features, hepatosplenomegaly, and developmental delay. The electroencephalogram and cranial ultrasound were abnormal, and a computed tomogram showed lissencephaly and apparent agenesis of the corpus callosum. Because of frequent aspiration she became oxygen dependent. She later develope...

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