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Atm-Deficient Mice: A Paradigm of Ataxia Telangiectasia
A murine model of ataxia telangiectasia was created by disrupting the Atm locus via gene targeting. Mice homozygous for the disrupted Atm allele displayed growth retardation, neurologic dysfunction,Expand
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A LIS1/NUDEL/Cytoplasmic Dynein Heavy Chain Complex in the Developing and Adult Nervous System
Mutations in mammalian Lis1 (Pafah1b1) result in neuronal migration defects. Several lines of evidence suggest that LIS1 participates in pathways regulating microtubule function, but the molecularExpand
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Graded reduction of Pafah1b1 (Lis1) activity results in neuronal migration defects and early embryonic lethality
Heterozygous mutation or deletion of the ß subunit of platelet-activating factor acetylhydrolase (PAFAH1B1, also known as LIS1) in humans is associated with type I lissencephaly, a severeExpand
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Neuroepithelial Stem Cell Proliferation Requires LIS1 for Precise Spindle Orientation and Symmetric Division
Mitotic spindle orientation and plane of cleavage in mammals is a determinant of whether division yields progenitor expansion and/or birth of new neurons during radial glial progenitor cell (RGPC)Expand
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LIS1 and NDEL1 coordinate the plus‐end‐directed transport of cytoplasmic dynein
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 demonstrateExpand
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Kinesin-1 regulates dendrite microtubule polarity in Caenorhabditis elegans
In neurons, microtubules (MTs) span the length of both axons and dendrites, and the molecular motors use these intracellular ‘highways' to transport diverse cargo to the appropriate subcellularExpand
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Complete Loss of Ndel1 Results in Neuronal Migration Defects and Early Embryonic Lethality
ABSTRACT Regulation of cytoplasmic dynein and microtubule dynamics is crucial for both mitotic cell division and neuronal migration. NDEL1 was identified as a protein interacting with LIS1, theExpand
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DISC1 regulates the transport of the NUDEL/LIS1/14-3-3epsilon complex through kinesin-1.
Disrupted-In-Schizophrenia 1 (DISC1) is a candidate gene for susceptibility to schizophrenia. DISC1 is reported to interact with NudE-like (NUDEL), which forms a complex with lissencephaly-1 (LIS1)Expand
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Hippocampal Abnormalities and Enhanced Excitability in a Murine Model of Human Lissencephaly
Human cortical heterotopia and neuronal migration disorders result in epilepsy; however, the precise mechanisms remain elusive. Here we demonstrate severe neuronal dysplasia and heterotopiaExpand
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Distinct Dose-Dependent Cortical Neuronal Migration and Neurite Extension Defects in Lis1 and Ndel1 Mutant Mice
Haploinsufficiency of LIS1 results in lissencephaly, a human neuronal migration disorder. LIS1 is a microtubule- (MT) and centrosome- [microtubule organizing center (MTOC)] associated protein thatExpand
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