نتایج جستجو برای: lissencephaly

تعداد نتایج: 686  

Journal: :Molecular biology and evolution 2015
Liang Liu Zhenxiang Xi Charles C Davis

It is well known that species with elevated substitution rates can give rise to disproportionately long branches in the species tree. This combination of long and short branches can contribute to long-branch artifacts (LBA). Despite efforts to remedy LBA via increased taxon sampling and methodological improvements in gene tree estimation, it remains unclear how long and short branches affect sp...

2005
BRIAN CONRAD

Suppose X is a locally noetherian Deligne–Mumford stack. Definition 1.2 has an obvious variant X̂ét using the underlying smaller étale site Xét and the restriction Oc Xét of Oc X to this site. By [3, 12.7.4], the category of cartesian Oc X -modules on Xlis-ét is equivalent to the category of Oc Xét-modules on Xét: (1.1) ModXlis-ét,cart(Oc X ) ' ModXét(Oc Xét) Definition 1.3. Let X be a locally n...

2011
Jinkyung Kim Namseop Kwon Soeun Chang Kyong-Tai Kim Dongmyeong Lee Seunghwan Kim So Jeong Yun Daehee Hwang Jee Woong Kim Yeukuang Hwu Giorgio Margaritondo Jung Ho Je Im Joo Rhyu

Disrupted cortical cytoarchitecture in cerebellum is a typical pathology in reeler. Particularly interesting are structural problems at the cellular level: dendritic morphology has important functional implication in signal processing. Here we describe a combinatorial imaging method of synchrotron X-ray microtomography with Golgi staining, which can deliver 3-dimensional(3-D) micro-architecture...

Journal: :The Biochemical journal 2005
Eva M Jiménez-Mateos Francisco Wandosell Orly Reiner Jesús Avila Christian González-Billault

For neuronal migration to occur, the cell must undergo morphological changes that require modifications of the cytoskeleton. Several different MAPs (microtubule-associated proteins) or actin-binding proteins are proposed to be involved in the migration of neurons. Therefore we have specifically analysed how two members of the MAP family, MAP1B and LIS1 (lissencephaly-related protein 1), interac...

Journal: :Neuron 2004
Tianzhi Shu Ramses Ayala Minh-Dang Nguyen Zhigang Xie Joseph G. Gleeson Li-Huei Tsai

Correct neuronal migration and positioning during cortical development are essential for proper brain function. Mutations of the LIS1 gene result in human lissencephaly (smooth brain), which features misplaced cortical neurons and disarrayed cerebral lamination. However, the mechanism by which LIS1 regulates neuronal migration remains unknown. Using RNA interference (RNAi), we found that the bi...

Journal: :Archives of ophthalmology 2012
Jennifer Hu Clement C Chow Daniel F Kiernan Enrique Garcia-Valenzuela Mahmood F Mafee Michael P Blair Michael J Shapiro

patient with West Nile virus infection. Arch Ophthalmol. 2003;121(2):205-207. 2. Garg S, Jampol LM. Systemic and intraocular manifestations of West Nile virus infection. Surv Ophthalmol. 2005;50(1):3-13. 3. Garg S, Jampol LM, Wilson JF, Batlle IR, Buettner H. Ischemic and hemorrhagic retinal vasculitis associated with West Nile virus infection. Retina. 2006; 26(3):365-367. 4. Chan CK, Limstrom ...

2003
Tatsushi Toda Kazuhiro Kobayashi Satoshi Takeda Junko Sasaki Hiroki Kurahashi Hiroki Kano Masaji Tachikawa Fan Wang Yoshitaka Nagai Kiyomi Taniguchi Mariko Taniguchi Yoshihide Sunada Toshio Terashima Tamao Endo Kiichiro Matsumura

Fukuyama-type congenital muscular dystrophy (FCMD), Walker-Warburg syndrome (WWS), and muscle-eye-brain (MEB) disease are clinically similar autosomal recessive disorders characterized by congenital muscular dystrophy, lissencephaly, and eye anomalies. We identified the gene for FCMD and MEB, which encodes the fukutin protein and the protein O-linked mannose β1, 2-N-acetylglucosaminyltransferas...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2017
Ida Signe Bohse Larsen Yoshiki Narimatsu Hiren Jitendra Joshi Lina Siukstaite Oliver J Harrison Julia Brasch Kerry M Goodman Lars Hansen Lawrence Shapiro Barry Honig Sergey Y Vakhrushev Henrik Clausen Adnan Halim

The cadherin (cdh) superfamily of adhesion molecules carry O-linked mannose (O-Man) glycans at highly conserved sites localized to specific β-strands of their extracellular cdh (EC) domains. These O-Man glycans do not appear to be elongated like O-Man glycans found on α-dystroglycan (α-DG), and we recently demonstrated that initiation of cdh/protocadherin (pcdh) O-Man glycosylation is not depen...

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