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Reduced mesencephalic astrocyte-derived neurotrophic factor expression by mutant androgen receptor contributes to neurodegeneration in a model of spinal and bulbar muscular atrophy pathology

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Neural Regeneration Research 2025年第9期20卷 2655-2666页

作者： Yiyang Qin Wenzhen Zhu Tingting Guo Yiran Zhang Tingting Xing Peng Yin Shihua li xiao-jiang li Su Yang Guangdong Key Laboratory of Non-human Primate Research Key Laboratory of CNS Regeneration(Ministry of Education)Guangdong–HongKong–Macao Institute of CNS RegenerationJinan UniversityGuangzhouGuangdong ProvinceChina

Spinal and bulbar muscular atrophy is a neurodegenerative disease caused by extended CAG trinucleotide repeats in the androgen receptor gene,which encodes a ligand-dependent transcription facto *** mutant androgen receptor protein,characterized by polyglutamine expansion,is prone to misfolding and forms aggregates in both the nucleus and cytoplasm in the brain in spinal and bulbar muscular atrophy *** aggregates alter protein-protein interactions and compromise transcriptional *** this study,we reported that in both cultured N2a cells and mouse brain,mutant androgen receptor with polyglutamine expansion causes reduced expression of mesencephalic astrocyte-de rived neurotrophic *** n of mesencephalic astrocyte-derived neurotrophic factor amelio rated the neurotoxicity of mutant androgen receptor through the inhibition of mutant androgen receptor *** down endogenous mesencephalic astrocyte-derived neurotrophic factor in the mouse brain exacerbated neuronal damage and mutant androgen receptor *** findings suggest that inhibition of mesencephalic astrocyte-derived neurotrophic factor expression by mutant androgen receptor is a potential mechanism underlying neurodegeneration in spinal and bulbar muscular atrophy.

关键词： androgen receptor mesencephalic astrocyte-derived neurotrophic factor mouse model neurodegeneration neuronal loss neurotrophic factor polyglutamine disease protein misfolding spinal and bulbar muscular atrophy transcription factor

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Influence of Species Differences on the Neuropathology of Transgenic Huntington's Disease Animal Models

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Journal of Genetics and Genomics 2012年第6期39卷 239-245页

作者： xiao-jiang li Shihua li Department of Human Genetics Emory University School of Medicine 615 Michael St 347 Atlanta Georgia 30322 USA Institute of Genetics and Developmental Biology Chinese Academy of Sciences No. 1 West Beichen Road Chaoyang District Beijing 100101 China

Transgenic animal models have revealed much about the pathogenesis of age-dependent neurodegenerative diseases and proved to be a useful tool for uncovering therapeutic targets. Huntington＇s disease is a well-characterized neurodegenerative disorder that is caused by expansion of a CAG repeat, which results in expansion of a polyglutamine tract in the N-terminal region of huntingtin （HTT）. Similar CAG/glutamine expansions are also found to cause eight other neurodegenerative diseases that affect distinct brain regions in an age- dependent manner. Identification of this CAG/glutamine expansion has led to the generation of a variety of transgenic animal models. Of these different animal models, transgenic mice have been investigated extensively, and they show similar neuropathology and phenotypes as seen in their respective diseases. The common pathological hallmark of age-dependent neurodegeneration is the formation of aggregates or inclusions consisting of misfolded proteins in the affected brain regions; however, overt or striking neurodegeneration and apoptosis have not been reported in most transgenic mouse models for age-dependent diseases, including HD. By comparing the neuropathology of transgenic HD mouse, pig, and monkey models, we found that mutant HTT is more toxic to larger animals than mice, and larger animals also show neuropathology that has not been uncovered by transgenic mouse models. This review will discuss the importance of transgenic large animal models for analyzing the treatments. pathogenesis of neurodegenerative diseases and developing effective

关键词： Aging Huntington's disease Neurodegeneration Species differences Polyglutamine Transgenic animals

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TALEN-mediated gene mutations in monkeys

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Neuroscience Bulletin 2014年第3期30卷 379-380页

作者： Yinghui Zheng xiao-jiang li State Key Laboratory of Molecular Developmental Biology Institute of Genetics and Developmental Biology Chinese Academy of Sciences

Genetic manipulation by targeting defined genomic loci allows the generation of precise mutations in the genome in animal models that can recapitulate genetic defects in human diseases caused by inherited or de novo mutations. However, most of the previous gene-targeting manipulations have been restricted to mice, because their embryonic stem cells are available and can be modulated at defined genomic loci. The recent discovery and application of sequence-specific endonucleases,

关键词： gene TALEN-mediated gene mutations in monkeys

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Beyond Mice:Genetically Modifying Larger Animals to Model Human Diseases

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Journal of Genetics and Genomics 2012年第6期39卷 237-238页

作者： xiao-jiang li Wei li Institute of Genetics and Developmental Biology Chinese Academy of SciencesBeijing 100101China Department of Human Genetics Emory University School of MedicineAtlantaGA 30322USA

Genetically modified animal models have been used widely to model human diseases for the purposes of investigating pathogenesis and developing therapeutic strategies. The genetic modification technology consists mainly of gene tar- geting and transgenic approaches, and genetic engineering to target genes in animals has now advanced to new technologies, such as zinc-finger nuclease （ZFN） and transcription activatorlike effector nuclease （TALEN）. However, most animals used for creating disease models are still mice or other small animals.

关键词： Genetically Modifying Animals Diseases

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New pathogenic insights from large animal models of neurodegenerative diseases

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Protein & Cell 2022年第10期13卷 707-720页

作者： Peng Yin Shihua li xiao-jiang li Weili Yang Guangdong Key Laboratory of Non-human Primate Research Guangdong-Hongkong-Macao Institute of CNS RegenerationJinan UniversityGuangzhou 510632China

Animal models are essential for investigating the pathogenesis and developing the treatment of human *** of genetic mutations responsible for neurodegenerative diseases has enabled the creation of a large number of small animal models that mimic genetic defects found in the affected *** the current animal models,rodents with genetic modifications are the most commonly used animal models and provided important insights into ***,most of genetically modified rodent models lack overt neurodegeneration,imposing challenges and obstacles in utilizing them to rigorously test the therapeutic effects on *** studies that used CRISPR/Cas9-targeted large animal(pigs and monkeys)have uncovered important pathological events that resemble neurodegeneration in the patient’s brain but could not be produced in small animal *** we highlight the unique nature of large animals to model neurodegenerative diseases as well as the limitations and challenges in establishing large animal models of neurodegenerative diseases,with focus on Huntington disease,Amyotrophic lateral sclerosis,and Parkinson *** also discuss how to use the important pathogenic insights from large animal models to make rodent models more capable of recapitulating important pathological features of neurodegenerative diseases.

关键词： large animal models neurodegenerative diseases CRISPR/Cas9

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A booming field of large animal model research

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Zoological Research 2024年第2期45卷 311-313页

作者： xiao-jiang li liangxue Lai Guangdong Key Laboratory of Non-human Primate Research Key Laboratory of CNS Regeneration(Ministry of Education)GHM Institute of CNS RegenerationJinan UniversityGuangzhouGuangdong 510632China State Key Laboratory of Bioactive Molecules and Druggability Assessment Jinan UniversityGuangzhouGuangdong 510632China CAS Key Laboratory of Regenerative Biology Guangdong Provincial Key Laboratory of Stem Cell and Regenerative MedicineGuangzhou Institutes of Biomedicine and HealthChinese Academy of SciencesGuangzhouGuangdong 510530China

Animal models are integral to the study of fundamental biological processes and the etiology of human *** animal models,especially those involving mice,have yielded abundant and significant insights,greatly enhancing ... 详细信息

关键词： mechanisms insight field

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CRISPR-Based Genome-Editing Tools for Huntington’s Disease Research and Therapy

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Neuroscience Bulletin 2022年第11期38卷 1397-1408页

作者： Yiyang Qin Shihua li xiao-jiang li Su Yang Guangdong Key Laboratory of Non-human Primate Research Guangdong-Hongkong-Macao Institute of CNS RegenerationJinan UniversityGuangzhou 510632China

Huntington’s disease(HD)is an autosomal dominantly-inherited neurodegenerative disease,which is caused by CAG trinucleotide expansion in exon 1 of the Huntingtin(HTT)*** HD is a rare disease,its monogenic nature makes it an ideal model in which to understand pathogenic mechanisms and to develop therapeutic strategies for neurodegenerative *** regularly-interspaced short palindromic repeats(CRISPR)is the latest technology for genome *** simple to use and highly efficient,CRISPR-based genome-editing tools are rapidly gaining popularity in biomedical research and opening up new avenues for disease ***,we review the development of CRISPR-based genome-editing tools and their applications in HD research to offer a translational perspective on advancing the genome-editing technology to HD treatment.

关键词： Huntington’s disease CRISPR Animal models

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Pathological insights from amyotrophic lateral sclerosis animal models:comparisons,limitations,and challenges

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Translational Neurodegeneration 2023年第1期12卷 242-257页

作者： Longhong Zhu Shihua li xiao-jiang li Peng Yin Guangdong Key Laboratory of Non-Human Primate Research Key Laboratory of CNS Regeneration(Ministry of Education)GHM Institute of CNS RegenerationJinan UniversityGuangzhou 510632China

In order to dissect amyotrophic lateral sclerosis(ALS),a multigenic,multifactorial,and progressive neurodegenerative disease with heterogeneous clinical presentations,researchers have generated numerous animal models to mimic the genetic *** and comparative analysis of these various models allows identification of the causes and mechanisms of ALS in order to finally obtain effective ***,most genetically modified rodent models lack overt pathological features,imposing challenges and limitations in utilizing them to rigorously test the potential *** studies using large animals,including pigs and non-human primates,have uncovered important events that resemble neurodegeneration in patients’brains but could not be produced in small *** we describe common features as well as discrepancies among these models,highlighting new insights from these ***,we will discuss how to make rodent models more capable of recapitulating important pathological features based on the important pathogenic insights from large animal models.

关键词： Amyotrophic lateral sclerosis Animal model Rodent Non-human primate

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Large animal models for Huntington's disease research

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Zoological Research 2024年第2期45卷 275-283页

作者： Bofeng Han Weien liang xiao-jiang li Shihua li Sen Yan Zhuchi Tu Guangdong-Hongkong-Macao CNS Regeneration Institute of Jinan University Key Laboratory of CNS Regeneration(Jinan University)-Ministry of EducationGuangdong Key Laboratory of Non-Human Primate ResearchGuangzhouGuangdong 510632China Department of Pathophysiology School of MedicineJinan UniversityGuangzhouGuangdong 510632China

Huntington'sdisease(HD)isahereditary neurodegenerative disorder for which there is currently no ***,the development of appropriate disease models is critical to thoroughly investigate disease *** genetic basis of HD involves the abnormal expansion of CAG repeats in the huntingtin(HTT)gene,leading to the expansion of a polyglutamine repeat in the HTT *** HTT carrying the expanded polyglutamine repeat undergoes misfolding and forms aggregates in the brain,which precipitate selective neuronal loss in specific brain *** models play an important role in elucidating the pathogenesis of neurodegenerative disorders such as HD and in identifying potential therapeutic *** to the marked species differences between rodents and larger animals,substantial efforts have been directed toward establishing large animal models for HD *** models are pivotal for advancing the discovery of novel therapeutic targets,enhancing effective drug delivery methods,and improving treatment *** have explored the advantages of utilizing large animal models,particularly pigs,in previous *** then,however,significant progress has been made in developing more sophisticated animal models that faithfully replicate the typical pathology of *** the current review,we provide a comprehensive overview of large animal models of HD,incorporating recent findings regarding the establishment of HD knock-in(KI)pigs and their genetic *** also explore the utilization of large animal models in HD research,with a focus on sheep,non-human primates(NHPs),and *** objective is to provide valuable insights into the application of these large animal models for the investigation and treatment of neurodegenerative disorders.

关键词： Huntington's disease Large animal models Sheep Non-human primates Transgenic pigs

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Genetically modified non-human primate models for research on neurodegenerative diseases

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Zoological Research 2024年第2期45卷 263-274页

作者： Ming-Tian Pan Han Zhang xiao-jiang li Xiang-Yu Guo Guangdong Key Laboratory of Non-human Primate Research Guangdong-Hongkong-Macao Institute of CNS RegenerationJinan UniversityGuangzhouGuangdong 510632China

Neurodegenerative diseases(NDs)are a group of debilitating neurological disorders that primarily affect elderly populations and include Alzheimer's disease(AD),Parkinson's disease(PD),Huntington's disease(HD),and amyotrophic lateral sclerosis(ALS).Currently,there are no therapies available that can delay,stop,or reverse the pathological progression of NDs in clinical *** the population ages,NDs are imposing a huge burden on public health systems and affected *** models are important tools for preclinical investigations to understand disease pathogenesis and test potential *** numerous rodent models of NDs have been developed to enhance our understanding of disease mechanisms,the limited success of translating findings from animal models to clinical practice suggests that there is still a need to bridge this translation *** World nonhuman primates(NHPs),such as rhesus,cynomolgus,and vervet monkeys,are phylogenetically,physiologically,biochemically,and behaviorally most relevant to *** is particularly evident in the similarity of the structure and function of their central nervous systems,rendering such species uniquely valuable for neuroscience ***,the development of several genetically modified NHP models of NDs has successfully recapitulated key pathologies and revealed novel *** review focuses on the efficacy of NHPs in modeling NDs and the novel pathological insights gained,as well as the challenges associated with the generation of such models and the complexities involved in their subsequent analysis.

关键词： Neurodegeneration Non-human primate Macaque monkey Animal model Gene modification

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