Results 41 to 50 of about 2,739 (166)
eLife, 2020 Heterozygous loss of human PAFAH1B1 (coding for LIS1) results in the disruption of neurogenesis and neuronal migration via dysregulation of microtubule (MT) stability and dynein motor function/localization that alters mitotic spindle orientation ...
Hyang Mi Moon +3 more
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Global developmental gene expression and pathway analysis of normal brain development and mouse models of human neuronal migration defects. [PDF]
PLoS Genetics, 2011 Heterozygous LIS1 mutations are the most common cause of human lissencephaly, a human neuronal migration defect, and DCX mutations are the most common cause of X-linked lissencephaly.
Tiziano Pramparo +7 more
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Lis1 Regulates Dynein as a Molecular Wedge [PDF]
Biophysical Journal, 2014 Cytoplasmic dynein walks along microtubules by coupling cycles of ATP hydrolysis in its motor domain with cycles of microtubule binding and release at its microtubule-binding domain. Unlike the other cytoskeletal motors--kinesin and myosin--that have achieved functional diversity through molecular diversity, a single isoform of cytoplasmic dynein is ...
Toropova, Katerina +5 more
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Developmental downregulation of LIS1 expression limits axonal extension and allows axon pruning
Biology Open, 2017 The robust axonal growth and regenerative capacities of young neurons decrease substantially with age. This developmental downregulation of axonal growth may facilitate axonal pruning and neural circuit formation but limits functional recovery following ...
Kanako Kumamoto +5 more
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iScience, 2022 Summary: In the axon terminal, microtubule stability is decreased relative to the axon shaft. The dynamic microtubule plus ends found in the axon terminal have many functions, including serving as a docking site for the Cytoplasmic dynein motor. Here, we
Dane Kawano +6 more
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Lissencephaly-1 dependent axonal retrograde transport of L1-type CAM Neuroglian in the adult drosophila central nervous system. [PDF]
PLoS ONE, 2017 Here, we established the Drosophila Giant Fiber neurons (GF) as a novel model to study axonal trafficking of L1-type Cell Adhesion Molecules (CAM) Neuroglian (Nrg) in the adult CNS using live imaging.
Sirisha R Kudumala +8 more
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Brain Sciences, 2021 The 17p13.3 chromosome region is often deleted or duplicated in humans, resulting in severe neurodevelopmental disorders such as Miller–Dieker syndrome (MDS) and 17p13.3 duplication syndrome.
Xiaonan Liu +3 more
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Mechanism Of Lis1 In Promoting Perineural Invasion Of Hnscc
International Dental JournalAim or purpose: This study investigates the role of LIS1 in promoting perineural invasion (PNI) and metastasis in HNSCC. Materials and methods: LIS1 expression was assessed in HNSCC using TCGA datasets. To evaluate the correlation between LIS1 and PNI in
Ao Dai, Lu Gao, Xiang yu Zhu
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Lissencephaly-1 is a context-dependent regulator of the human dynein complex
eLife, 2017 The cytoplasmic dynein-1 (dynein) motor plays a central role in microtubule organisation and cargo transport. These functions are spatially regulated by association of dynein and its accessory complex dynactin with dynamic microtubule plus ends. Here, we
Janina Baumbach +6 more
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The Long Haul: Microtubule Motors as the Essential Supply Line for Neuronal Longevity
Journal of Neurochemistry, Volume 170, Issue 6, June 2026.To survive a lifetime, neurons depend on a high‐fidelity logistics network powered by microtubule motors. We explore how a broad spectrum of genetic defects in this machinery drive a devastating spectrum of neurodevelopmental and neurodegenerative diseases, including Hereditary Spastic Paraplegia (HSP), Charcot–Marie‐Tooth Type 2 (CMT2), and ...
Emma D. Turner, Alison E. Twelvetrees
wiley +1 more source