Explanipedia

Author Correction: Mitochondrial respiratory complex IV deficiency recapitulates amyotrophic lateral sclerosis Open

Cheng Man, Dan Lü, Kaihui Li, Yizhen Wang, Xiwen Tong , et al. · 2025

Sirt6 Mono‐ADP‐Ribosylates YY1 to Promote Dystrophin Expression for Neuromuscular Transmission Open

Wei Zhang, Lei Bai, Wentao Xu, Junping Liu, Yi Chen , et al. · 2024

The degeneration of the neuromuscular junction (NMJ) and the decline in motor function are common features of aging, but the underlying mechanisms have remained largely unclear. This study reveals that Sirt6 is reduced in aged mouse muscle…

Local protein synthesis at neuromuscular synapses is required for motor functions Open

Wen-Yo Tu, Wentao Xu, Lei Bai, Jun Liu, Yi Han , et al. · 2024

Motor neurons are highly polarized, and their axons extend over great distances to form connections with myofibers via neuromuscular junctions (NMJs). Local translation at the NMJs in vivo has not been identified. Here, we utilized motor n…

Negative regulation of TREM2-mediated C9orf72 poly-GA clearance by the NLRP3 inflammasome Open

Xiaoqiu Shu, Wei Chen, Wen-Yo Tu, Keke Zhong, Shuyuan Qi , et al. · 2023

Expansion of the hexanucleotide repeat GGGGCC in the C9orf72 gene is the most common genetic factor in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Poly-Gly-Ala (poly-GA), one form of dipeptide repeat proteins (DP…

C9orf72 poly-GA proteins impair neuromuscular transmission Open

Wen-Yo Tu, Wentao Xu, Jianmin Zhang, Shuyuan Qi, Lei Bai , et al. · 2023

Amyotrophic lateral sclerosis (ALS) is a devastating motoneuron disease, in which lower motoneurons lose control of skeletal muscles. Degeneration of neuromuscular junctions (NMJs) occurs at the initial stage of ALS. Dipeptide repeat prote…

Motoneurons innervation determines the distinct gene expressions in multinucleated myofibers Open

Lei Bai, Wen-Yo Tu, Yatao Xiao, Kejing Zhang, Chengyong Shen · 2022

Human neuromuscular junction three-dimensional organoid models and the insight in motor disorders Open

Kejing Zhang, Lei Bai, Wentao Xu, Chengyong Shen · 2021

The neuromuscular junction (NMJ), a peripheral synaptic connection between motoneurons and skeletal muscle fibers, controls movement. Dysregulation of NMJs has been implicated in various motor disorders. Because of their large size and eas…

UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model Open

Kejing Zhang, Wang Ailian, Keke Zhong, Shuyuan Qi, Wei Chen , et al. · 2021

Sarcoglycan Alpha Mitigates Neuromuscular Junction Decline in Aged Mice by Stabilizing LRP4 Open

Kai Zhao, Chengyong Shen, Lei Li, Haitao Wu, Guanglin Xing , et al. · 2018

During aging, acetylcholine receptor (AChR) clusters become fragmented and denervated at the neuromuscular junction (NMJ). Underpinning molecular mechanisms are not well understood. We showed that LRP4, a receptor for agrin and critical fo…

Motoneuron Wnts regulate neuromuscular junction development Open

Chengyong Shen, Lei Li, Kai Zhao, Lei Bai, Ailian Wang , et al. · 2018

The neuromuscular junction (NMJ) is a synapse between motoneurons and skeletal muscles to control motor behavior. Unlike extensively investigated postsynaptic differentiation, less is known about mechanisms of presynaptic assembly. Genetic…

Author response: Motoneuron Wnts regulate neuromuscular junction development Open

Chengyong Shen, Lei Li, Kai Zhao, Lei Bai, Ailian Wang , et al. · 2018

Muscle Yap Is a Regulator of Neuromuscular Junction Formation and Regeneration Open

Kai Zhao, Chengyong Shen, Yisheng Lu, Zhihui Huang, Lei Li , et al. · 2017

Yes-associated protein (Yap) is a major effector of the Hippo pathway that regulates cell proliferation and differentiation during development and restricts tissue growth in adult animals. However, its role in synapse formation remains poo…

Enzymatic Activity of the Scaffold Protein Rapsyn for Synapse Formation Open

Lei Li, Yu Cao, Haitao Wu, Xinchun Ye, Zhihui Zhu , et al. · 2016

Agrin and low-density lipoprotein-related receptor protein 4 antibodies in amyotrophic lateral sclerosis patients Open

Michael H. Rivner, Siyang Liu, Brandy Quarles, Brandi Fleenor, Chengyong Shen , et al. · 2016

Antibodies to agrin and LRP4 are found in ALS patients. It must be determined whether these antibodies are pathogenic. Because antibody-positive patients have upper as well as lower motor neuron findings, the antibodies' effects cannot be …

Lrp4 in astrocytes modulates glutamatergic transmission Open

Xiang-Dong Sun, Lei Li, Fang Liu, Zhi-Hui Huang, Jonathan C. Bean , et al. · 2016

Flow Cytofluorimetric Analysis of Anti-LRP4 (LDL Receptor-Related Protein 4) Autoantibodies in Italian Patients with Myasthenia Gravis Open

Mariapaola Marino, Flavia Scuderi, Daniela Samengo, Giorgia Saltelli, Maria Teresa Maiuri , et al. · 2015

Our data confirm LRP4 as a new autoimmune target, supporting the value of including anti-LRP4 antibodies in further studies on Myasthenia gravis.

Slit2 as a β-catenin/Ctnnb1-dependent retrograde signal for presynaptic differentiation Open

Haitao Wu, Arnab Barik, Yisheng Lu, Chengyong Shen, Andrew Bowman , et al. · 2015

Neuromuscular junction formation requires proper interaction between motoneurons and muscle cells. β-Catenin (Ctnnb1) in muscle is critical for motoneuron differentiation; however, little is known about the relevant retrograde signal. In t…

Author response: Slit2 as a β-catenin/Ctnnb1-dependent retrograde signal for presynaptic differentiation Open

Haitao Wu, Arnab Barik, Yisheng Lu, Chengyong Shen, Andrew Bowman , et al. · 2015

Article Figures and data Abstract eLife digest Introduction Results Discussion Materials and methods References Decision letter Author response Article and author information Metrics Abstract Neuromuscular junction formation requires prope…

Lrp4 in osteoblasts suppresses bone formation and promotes osteoclastogenesis and bone resorption Open

Lei Xiong, Ji-Ung Jung, Haitao Wu, Wen-Fang Xia, Jin‐Xiu Pan , et al. · 2015

Significance This study provides in vivo evidence that Lrp4 loss in osteoblast (OB)-lineage cells results in high–bone-mass deficit. Lrp4 in OB-lineage cells is required to reduce serum levels of sclerostin and to facilitate sclerostin inh…

ERBB3-mediated regulation of Bergmann glia proliferation in cerebellar lamination Open

Anupama Sathyamurthy, Dong‐Min Yin, Arnab Barik, Chengyong Shen, Jonathan C. Bean , et al. · 2015

Cortical lamination is crucial for the assembly of cerebellar circuitry. In this process, granule neurons (GNs) migrate along Bergmann glia (BG), which are specialized astroglial cells, from the external granule layer to the internal granu…

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