نتایج جستجو برای: lissencephaly

تعداد نتایج: 686  

Journal: :American journal of human genetics 2000
J G Gleeson S Minnerath R I Kuzniecky W B Dobyns I D Young M E Ross C A Walsh

Mutations in the X-linked gene doublecortin lead to "double cortex" syndrome (DC) in females and to X-linked lissencephaly (XLIS) in males. Because most patients with DC and XLIS are sporadic, representing de novo doublecortin mutations, we considered that some of these patients could be somatic or germline mosaics. Among a population of 20 patients and their families, we found evidence for mos...

Journal: :Learning & memory 1999
R Paylor S Hirotsune M J Gambello L Yuva-Paylor J N Crawley A Wynshaw-Boris

Heterozygous mutation or deletion of Pafab1b1 (LIS1) in humans is associated with syndromes with type 1 lissencephaly, a severe brain developmental disorder resulting from abnormal neuronal migration. We have created Lis1 heterozygous mutant mice by gene targeting. Heterozygous mutant mice are viable and fertile, but display global organizational brain defects as a result of impaired neuronal m...

Journal: :European Psychiatry 2023

Introduction The deductive method: from karyotyping to aCGH and WES is an important aspect in the diagnosis search for causes of intellectual disability due congenital brain anomalies. There recommendation exclude presence CNV or monogenic variants patients with a normal karyotype, but clinical picture syndromic disease. Objectives Improvement disability. Methods 60K Agilent microarrays, SureSe...

Journal: :Wiley Interdisciplinary Reviews: Developmental Biology 2012

Journal: :American Journal of Medical Genetics Part B: Neuropsychiatric Genetics 2006

Journal: :Brain : a journal of neurology 2013
Thomas D Cushion William B Dobyns Jonathan G L Mullins Neil Stoodley Seo-Kyung Chung Andrew E Fry Ute Hehr Roxana Gunny Arthur S Aylsworth Prab Prabhakar Gökhan Uyanik Julia Rankin Mark I Rees Daniela T Pilz

Polymicrogyria and lissencephaly are causally heterogeneous disorders of cortical brain development, with distinct neuropathological and neuroimaging patterns. They can be associated with additional structural cerebral anomalies, and recurrent phenotypic patterns have led to identification of recognizable syndromes. The lissencephalies are usually single-gene disorders affecting neuronal migrat...

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