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Clinical and genetic heterogeneity of adult polyglucosan body disease caused by GBE1 biallelic mutations in China

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Genes & Diseases 2024年第5期11卷 17-20页

作者： Yikun Chen Yan Shi Yuan Gao Yan Hu Linying Zhou Jingmei Hong Shirui Gan Xiang Lin Wanjin Chen Guorong Xu Jin He Department of Neurology and institute of Neurology of First Affiliated Hospital Institute of Neuroscienceand Fujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian 350005China Fujian Key Laboratory of Molecular Neurology Institute of NeuroscienceFujian Medical UniversityFuzhouFujian 350005China Electron Microscopy Lab of Public Technology Service Center Fujian Medical UniversityFuzhouFujian 350005China

Adult polyglucosan body disease(APBD)is a rare and highly heterogeneous glycogen storage disorder due to biallelic variants in GBE1.1 Typical APBD presentations include gait abnormalities with polyneuropathy,leukodystrophy,neurogenic bladder,and mild cognitive *** diagnosis of APBD encompasses a large spectrum of conditions including axonal and demyelinating sensorimotor polyneuropathy,progressive spastic paraparesis,and leukodystrophies.

关键词： GBE bladder diagnosis

Does astrocytic L-lactate enhance cognition through myelination?

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Neural Regeneration Research 2024年第6期19卷 1167-1168页

作者： Mastura Akter Ying Li Department of neuroscience City University of Hong Kong Department of Biomedical Sciences City Universit of Hong Kong Centre for Regenerative Medicine and Health Hong Kong Institute of Science & Innovation Chinese Academy of Sciences Centre for Biosystems Neuroscience and Nanotechnology City University of Hong Kong

Introduction: Astrocytes, the predominant glial cell in the brain, play a vital role in a plethora of central ner vous system functions. They are the major storage site of glycogen in the central nervous system. They produce L-lactate by glycogenolysis and glycolysis which is then transported to neurons(Magistreti and Allaman,

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The bumpy road of purinergic inhibitors to clinical application in immune-mediated diseases

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Neural Regeneration Research 2024年第6期19卷 1206-1211页

作者： Matihias T.Wyss Christine Heuer Marina Herwerth institute of Pharmacology and Toxicology University of Zurich neuroscience Center Zurich (ZNZ) University of Zurich and ETH Zurich 3. Neurology Department University Hospital of Zurich

Purinergic signaling plays important roles throughout the body in the regulation of organ functions during and following the disruption of homeostasis. This is also reflected by the widespread expression of two families of purinergic receptors(P1 and P2) with numerous subtypes. In the last few decades, there has been increasing evidence that purinergic signaling plays an important role in the regulation of immune functions. Mainly, signals mediated by P2 receptors have been shown to contribute to immune system-mediated pathologies. Thus, interference with P2 receptors may be a promising strategy for the modulation of immune responses. Although only a few clinical studies have been conducted in isolated entities with limited success, preclinical work suggests that the use of P2 receptor inhibitors may bear some promise in various autoimmune diseases. Despite the association of P2 receptors with several disorders from this field, the use of P2 receptor antagonists in clinical therapy is still very scarce. In this narrative review, we briefly review the involvement of the purinergic system in immunological responses and clinical studies on the effect of purinergic inhibition on autoimmune processes. We then open the aperture a bit and show some preclinical studies demonstrating a potential effect of purinergic blockade on autoimmune events. Using suramin, a non-specific purinergic inhibitor, as an example, we further show that off-target effects could be responsible for observed effects in immunological settings, which may have interesting implications. Overall, we believe that it is worthwhile to further investigate this hitherto underexplored area.

关键词： autoimmune diseases neurological disorders purinergic system P2 receptor inhibitors suramin

TET1 Participates in Complete Freund’s Adjuvant-induced Trigeminal Inflammatory Pain by Regulating Kv7.2 in a Mouse Model

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neuroscience Bulletin 2024年第6期40卷 707-718页

作者： Dengcheng Zhan Jingjing Zhang Songxue Su Xiuhua Ren Sen Zhao Weidong Zang Jing Cao Department of Human Anatomy School of Basic Medical SciencesZhengzhou UniversityZhengzhou450001China neuroscience Research institute Zhengzhou UniversityZhengzhou450001China

Trigeminal inflammatory pain is one of the most severe pain-related disorders in humans;however,the underlying mechanisms remain largely *** this study,we investigated the possible contribution of interaction between ten-eleven translocation methylcytosine dioxygenase 1(TET1)and the voltage-gated K^(+)channel Kv7.2(encoded by Kcnq2)to orofacial inflammatory pain in *** found that complete Freund’s adjuvant(CFA)injection reduced the expression of Kcnq2/Kv7.2 in the trigeminal ganglion(TG)and induced orofacial inflammatory *** involvement of Kv7.2 in CFA-induced orofacial pain was further confirmed by Kv7.2 knockdown or ***,TET1 knockdown in Tet1^(flox/flox)mice significantly reduced the expression of Kv7.2 and M currents in the TG and led to pain-like ***,TET1 overexpression by lentivirus rescued the CFA-induced decreases of Kcnq2 and M currents and alleviated mechanical *** data suggest that TET1 is implicated in CFA-induced trigeminal inflammatory pain by positively regulating Kv7.2 in TG neurons.

关键词： Facial pain KCNQ2 potassium channel TET1 protein

Connecting neurodevelopment to neurodegeneration:a spotlight on the role of kinesin superfamily protein 2A(KIF2A)

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Neural Regeneration Research 2024年第2期19卷 375-379页

作者： Nuria Ruiz-Reig Janne Hakanen Fadel Tissir Universitécatholique de Louvain Institute of neuroscienceBrusselsBelgium College of Health and Life Sciences Hamad Bin Khalifa University(HBKU)DohaQatar

Microtubules play a central role in cytoskeletal changes during neuronal development and *** dynamics is essential to polarity and shape transitions underlying neural cell division,differentiation,motility,and *** superfamily protein 2A is a member of human kinesin 13 gene family of proteins that depolymerize and destabilize *** dividing cells,kinesin superfamily protein 2A is involved in mitotic progression,spindle assembly,and chromosome *** postmitotic neurons,it is required for axon/dendrite specification and extension,neuronal migration,connectivity,and *** with kinesin superfamily protein 2A mutations suffer from a variety of malformations of cortical development,epilepsy,autism spectrum disorder,and *** this review,we discuss how kinesin superfamily protein 2A regulates neuronal development and function,and how its deregulation causes neurodevelopmental and neurological disorders.

关键词： brain disorders cortical malformations kinesin microtubules neurodegeneration neurodevelopment

Implications of regional identity for neural stem and progenitor cell transplantation in the injured or diseased nervous system

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Neural Regeneration Research 2024年第4期19卷 715-716页

作者： Prakruthi Amar Kumar Jennifer N.Dulin Department of Biology Texas A&M UniversityCollege Station Texas A&M institute for neuroscience Texas A&M UniversityCollege Station

Neural stem and progenitor cell(NSPC)transpla ntation has emerged as a promising therapeutic strategy for replacing lost neuronal populations and repairing damaged neural circuits following nervous system injury and disease.A great deal of experimental work has investigated the biology of NSPC grafting in preclinical animal models;more recently.

关键词： neural system injured

Deformable microlaser force sensing

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Light(Science & Applications) 2024年第6期13卷 1196-1209页

作者： Eleni Dalaka Joseph SHill Jonathan H.H.Booth Anna Popczyk Stefan R.Pulver Malte C.Gather Marcel Schubert Centre of Biophotonics SUPASchool of Physics and AstronomyUniversity of St AndrewsNorth HaughSt AndrewsUK Humboldt Centre for Nano-and Biophotonics Department of ChemistryUniversity of CologneKölnGermany School of Psychology and neuroscience University of St AndrewsSt Mary’s QuadSouth StreetSt AndrewsUK institute for Bioengineering of Catalonia BarcelonaSpain

Mechanical forces are key regulators of cellular behavior and function,affecting many fundamental biological processes such as cell migration,embryogenesis,immunological responses,and pathological *** force sensors and imaging techniques have been developed to quantify these otherwise invisible forces in single cells and in ***,current techniques rely heavily on high-resolution microscopy and do not allow interrogation of optically dense tissue,reducing their application to 2D cell cultures and highly transparent biological ***,we introduce DEFORM,deformable microlaser force sensing,a spectroscopic technique that detects sub-nanonewton forces with unprecedented spatio-temporal *** is based on the spectral analysis of laser emission from dye-doped oil microdroplets and uses the force-induced lifting of laser mode degeneracy in these droplets to detect nanometer *** validation by atomic force microscopy and development of a model that links changes in laser spectrum to applied force,DEFORM is used to measure forces in 3D and at depths of hundreds of microns within tumor spheroids and late-stage Drosophila *** furthermore show continuous force sensing with single-cell spatial and millisecond temporal resolution,thus paving the way for non-invasive studies of biomechanical forces in advanced stages of embryogenesis,tissue remodeling,and tumor invasion.

关键词： invasion otherwise hundreds

Advancing neuroscience through real-time processing of big data:Transition from open-loop to closed-loop paradigms

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Zoological Research 2024年第3期45卷 518-519页

作者： Yu-Fan Wang Jiu-Lin Du institute of neuroscience State Key Laboratory of NeuroscienceCenter for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China University of Chinese Academy of Sciences Beijing 100049China School of Life Science and Technology ShanghaiTech UniversityShanghai 200031China

The brain functions as a closed-loop system that continuously generates behavior in response to the external environment and adjusts actions based on the *** research methodologies in neuroscience,especially those employed in brain imaging experiments,have mainly adopted an open-loop paradigm(Grosenick et al.,2015).Functional neural circuits are analyzed offline and subsequently tested through manipulation of neuronal activities within specific regions or with genetic *** establishing a closed-loop research paradigm,functional ensembles can be detected and tested in real time with temporal *** functional ensembles,rather than brain regions or genetically labeled neural populations,serve as fundamental units of neural networks,offering valuable insights into the dissection of neural *** closed-loop research paradigm also enables the capture of high-dimensional activities of internal brain dynamics and precise elucidation of physiological processes such as learning,decision-making,and sleep.

关键词： neural loop Transition

Generation of mitochondrial replacement monkeys by female pronucleus transfer

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Zoological Research 2024年第2期45卷 292-298页

作者： Chun-Yang Li Xing-Chen Liu Yu-Zhuo Li Yan Wang Yan-Hong Nie Yu-Ting Xu Xiao-Tong Zhang Yong Lu Qiang Sun institute of neuroscience Center for Excellence in Brain Science and Intelligence TechnologyState Key Laboratory of NeuroscienceChinese Academy of SciencesShanghai 200031China Key Laboratory of Genetic Evolution and Animal Models Chinese Academy of SciencesKunming 650201China University of Chinese Academy of Sciences Beijing 100049China

Mutations in mitochondrial DNA(mtDNA)are maternally inherited and have the potential to cause severe *** replacement therapies,including spindle,polar body,and pronuclear transfers,are promising strategies for preventing the hereditary transmission of mtDNA *** pronuclear transfer has been used to generate mitochondrial replacement mouse models and human embryos,its application in non-human primates has not been previously *** this study,we successfully generated four healthy cynomolgus monkeys(Macaca fascicularis)via female pronuclear *** individuals all survived for more than two years and exhibited minimal mtDNA carryover(3.8%–6.7%),as well as relatively stable mtDNA heteroplasmy dynamics during *** successful establishment of this nonhuman primate model highlights the considerable potential of pronuclear transfer in reducing the risk of inherited mtDNA diseases and provides a valuable preclinical research model for advancing mitochondrial replacement therapies in humans.

关键词： Non-human primates Mitochondrial replacement Female pronuclear transfer