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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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Differential distribution of PINK1 and Parkin in the primate brain implies distinct roles

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Neural Regeneration research 2025年第4期20卷 1124-1134页

作者： Yanting Liu Wei Huang Jiayi Wen Xin Xiong Ting Xu Qi Wang Xiusheng Chen Xianxian Zhao Shihua Li Xiaojiang Li Weili Yang guangdong key laboratory of non-human primate research Key Laboratory of CNS Regeneration(Ministry of Education)Guangdong-Hongkong-Macao CNS Regeneration Institute of Jinan UniversityJinan UniversityGuangzhouGuangdong ProvinceChina

The vast majority of in vitro studies have demonstrated that PINK1 phosphorylates Parkin to work together in mitophagy to protect against neuronal ***,it remains largely unclear how PINK1 and Parkin are expressed in mammalian *** has been difficult to address because of the intrinsically low levels of PINK1 and undetectable levels of phosphorylated Parkin in small *** this issue is critical for elucidating the in vivo roles of PINK1 and ***,we showed that the PINK1 kinase is selectively expressed as a truncated form(PINK1–55)in the primate *** the present study,we used multiple antibodies,including our recently developed monoclonal anti-PINK1,to validate the selective expression of PINK1 in the primate *** found that PINK1 was stably expressed in the monkey brain at postnatal and adulthood stages,which is consistent with the findings that depleting PINK1 can cause neuronal loss in developing and adult monkey ***1 was enriched in the membrane-bound fractionations,whereas Parkin was soluble with a distinguishable *** double staining experiments showed that PINK1 and Parkin did not colocalize under physiological conditions in cultured monkey astrocytes,though they did colocalize on mitochondria when the cells were exposed to mitochondrial *** findings suggest that PINK1 and Parkin may have distinct roles beyond their well-known function in mitophagy during mitochondrial damage.

关键词： neurodegeneration Parkin Parkinson’s disease PINK1 subcellular distribution

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Advances and applications of genome-edited animal models for severe combined immunodeficiency

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Zoological research 2025年第01期 249-260页

作者： Xiao Zheng Chun-Hui Huang Sen Yan Ming-Deng Rong Stomatological Hospital School of Stomatology Southern Medical University guangdong key laboratory of non-human primate Models Guangdong-Hongkong-Macau Institute of CNS Regeneration Jinan University School of medicine Jinan University Department of Neurology Guangzhou Red Cross Hospital Faculty of Medical Science Jinan University

Severe combined immunodeficiency disease(SCID),characterized by profound immune system dysfunction,can lead to life-threatening infections and death. Animal models play a pivotal role in elucidating biological processes and advancing therapeutic strategies. Recent advances in gene-editing technologies, including zincfinger nucleases(ZFNs), transcription activator-like effector nucleases(TALENs), CRISPR/Cas9, and base editing, have significantly enhanced the generation of SCID models. These models have not only deepened our understanding of disease pathophysiology but have also driven progress in cancer therapy, stem cell transplantation, organ transplantation, and infectious *** comprehensive overview of current SCID models generated using novel gene-editing approaches,highlighting their potential applications in translational medicine and their role in advancing biomedical research.

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Assessment of internal exposure risk from metals pollution of occupational and non-occupational populations around a non-ferrous metal smelting plant

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Journal of Environmental Sciences 2025年第1期147卷 62-73页

作者： Yingjun Wu Mengdie Qi Hang Yu Guiying Li Taicheng An guangdong-Hong Kong-Macao Joint laboratory for Contaminants Exposure and Health Guangdong Key Laboratory of Environmental Catalysis and Health Risk ControlInstitute of Environmental Health and Pollution ControlGuangdong University of TechnologyGuangzhou 510006China Guangzhou key laboratory of Environmental Catalysis and Pollution Control Key Laboratory for City Cluster Environmental Safety and Green DevelopmentSchool of Environmental Science and EngineeringGuangdong University of TechnologyGuangzhou 510006China

non-ferrous metal smelting poses significant risks to public ***,the copper smelting process releases arsenic,a semi-volatile metalloid,which poses an emerging exposure risk to both workers and nearby *** comprehensively understand the internal exposure risks of metal(loid)s from copper smelting,we explored eighteen metal(loid)s and arsenic metabolites in the urine of both occupational and non-occupational populations using inductively coupled plasma mass spectrometry with high-performance liquid chromatography and compared their health *** showed that zinc and copper(485.38 and 14.00μg/L),and arsenic,lead,cadmium,vanadium,tin and antimony(46.80,6.82,2.17,0.40,0.44 and 0.23μg/L,respectively)in workers(n=179)were significantly higher compared to controls(n=168),while Zinc,tin and antimony(412.10,0.51 and 0.15μg/L,respectively)of residents were significantly higher than ***,workers had a higher monomethyl arsenic percentage(MMA%),showing lower arsenic methylation *** appointment analysis identified arsenic,lead,cadmium,antimony,tin and thallium as co-exposure metal(loid)s from copper smelting,positively relating to the age of *** hazard index(HI)of workers exceeded 1.0,while residents and control were approximately at ***,all three populations had accumulated cancer risks exceeding 1.0×10^(-4),and arsenite(AsIII)was the main contributor to the variation of workers and ***,residents living closer to the smelting plant had higher health *** study reveals arsenic exposure metabolites and multiple metals as emerging contaminants for copper smelting exposure populations,providing valuable insights for pollution control in non-ferrous metal smelting.

关键词： Arsenic metabolites Metal(loid)s non-ferrous metal smelting human biomonitoring Exposure Urine

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The microbiome's influence on obesity: mechanisms and therapeutic potential

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Science China Life Sciences 2025年第3期 657-672页

作者： Dawit Adisu Tadese James Mwangi Lei Luo Hao Zhang Xiaoshan Huang Brenda B.Michira Shengwen Zhou Peter Muiruri Kamau Qiumin Lu Ren Lai Engineering laboratory of Peptides of Chinese Academy of Sciences Key Laboratory of Bioactive Peptides of Yunnan Province KIZ-CUHK Joint Laboratory of Bioresources and Molecular Research in Common Diseases National Resource Center for Non-Human Primates National Research Facility for Phenotypic & Genetic Analysis of Model Animals (Primate Facility) Key Laboratory of Genetic Evolution & Animal Models Sino-African Joint Research Center and New Cornerstone Science Laboratory Kunming Institute of Zoology Chinese Academy of Sciences Kunming College of Life Sciences University of Chinese Academy of Sciences

In 2023, the World Obesity Atlas Federation concluded that more than 50% of the world's population would be overweight or obese within the next 12 years. At the heart of this epidemic lies the gut microbiota, a complex ecosystem that profoundly influences obesity-related metabolic health. Its multifaced role encompasses energy harvesting, inflammation, satiety signaling, gut barrier function, gut-brain communication, and adipose tissue homeostasis. Recognizing the complexities of the cross-talk between host physiology and gut microbiota is crucial for developing cutting-edge, microbiome-targeted therapies to address the global obesity crisis and its alarming health and economic repercussions. This narrative review analyzed the current state of knowledge, illuminating emerging research areas and their implications for leveraging gut microbial manipulations as therapeutic strategies to prevent and treat obesity and related disorders in humans. By elucidating the complex relationship between gut microflora and obesity, we aim to contribute to the growing body of knowledge underpinning this critical field, potentially paving the way for novel interventions to combat the worldwide obesity epidemic.

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Drosophila models used to simulate human ATP1A1 gene mutations that cause Charcot-Marie-Tooth type 2 disease and refractory seizures

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Neural Regeneration research 2025年第1期20卷 265-276页

作者： Yao Yuan Lingqi Yu Xudong Zhuang Dongjing Wen Jin He Jingmei Hong Jiayu Xie Shengan Ling Xiaoyue Du Wenfeng Chen Xinrui Wang Institute of Life Sciences College of Biological Science and EngineeringFuzhou UniversityFuzhouFujian ProvinceChina NHC key laboratory of Technical Evaluation of Fertility Regulation for non-human primate(Fujian Maternity and Child Health Hospital) FuzhouFujian ProvinceChina Medical research Center Fujian Maternity and Child Health HospitalCollege of Clinical Medicine for Obstetrics&Gynecology and PediatricsFujian Medical UniversityFuzhouFujian ProvinceChina Department of Neurology and Institute of Neurology of The First Affiliated Hospital Institute of Neuroscienceand Fujian Key Laboratory of Molecular NeurologyFujian Medical UniversityFuzhouFujian ProvinceChina

Certain amino acids changes in the human Na^(+)/K^(+)-ATPase pump,ATPase Na^(+)/K^(+)transporting subunit alpha 1(ATP1A1),cause Charcot-Marie-Tooth disease type 2(CMT2)disease and refractory *** develop in vivo models to study the role of Na^(+)/K^(+)-ATPase in these diseases,we modified the Drosophila gene homolog,Atpα,to mimic the human ATP1A1 gene mutations that cause *** located within the helical linker region of human ATP1A1(I592T,A597T,P600T,and D601F)were simultaneously introduced into endogenous Drosophila Atpαby CRISPR/Cas9-mediated genome editing,generating the Atpα^(TTTF)*** addition,the same strategy was used to generate the corresponding single point mutations in flies(Atpα^(I571T),Atpα^(A576T),Atpα^(P579T),and Atpα^(D580F)).Moreover,a deletion mutation(Atpα^(mut))that causes premature termination of translation was generated as a positive *** these alleles,we found two that could be maintained as homozygotes(Atpα^(I571T)and Atpα^(P579T)).Three alleles(Atpα^(A576T),Atpα^(P579)and Atpα^(D580F))can form heterozygotes with the Atpαmut *** found that the Atpαallele carrying these CMT2-associated mutations showed differential phenotypes in *** heterozygous for Atpα^(TTTF)mutations have motor performance defects,a reduced lifespan,seizures,and an abnormal neuronal *** Drosophila models will provide a new platform for studying the function and regulation of the sodium-potassium pump.

关键词： ATP1A1 Atpα bang-sensitive paralysis Charcot-Marie-Tooth disease type 2 CRISPR/Cas9 homology-directed repair Na^(+)/K^(+)-ATPase point mutation seizures sodium pump

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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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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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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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