ArticleCell2018
A Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington's Disease.
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Article in Cell, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 183 papers, 1 of them a synthesis that pooled it.
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183citing papers in PubMed, 1 pooled it
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Who cites it
183 citing papers in PubMed, 1 synthesis or guideline pooled it, 332 citations in OpenAlex.
- Neurodevelopmental Clues to Neurodegeneration.Pediatric neurology · 2021Pooled it
- CRISPR/Cas9-based nanodelivery systems for precision gene editing in neuroinflammation.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026Review
- A single-cell profile of the porcine immune repertoire: concurrent profiling of T cell receptor and B cell receptor V(D)J sequences in health.Advanced biotechnology · 2026Article
- Species-dependent activities of the PINK1-parkin axis.Translational neurodegeneration · 2026Review
- TH/TRs-COL11A2 Axis Mediates Loss of a Differentiated Astrocyte State in Hypogyrified Brains.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Development of mitochondrial mutator pigs exhibiting increased mitochondrial DNA mutations and premature aging via prime editing.Science advances · 2026Article
- Bridging precision agriculture and human medicine through comparative genetics.Nature reviews. Genetics · 2026Review
- Pig Matrix: a matched multiomics 3D regulatory genomics database for evolutionary and comparative analyses in pigs.Molecular biology and evolution · 2026Article
- CRISPR-Cas9-based therapies for Huntington's disease and Friedreich's ataxia: mechanisms, advances, and future perspectives.Neurogenetics · 2026Review
- Integrated ATAC-Seq and RNA-Seq Reveal Candidate Regulatory Genes and Chromatin Accessibility Associated with Intramuscular Fat Deposition: An Animal Trial in Hezuo Pigs.Animals : an open access journal from MDPI · 2026Article
- PAM-flexible SpCas9 variants expand the targeting scope for porcine genome editing and cellular disease modeling.BMC biotechnology · 2026Article
- Molecular features of a Huntington's disease knock-in minipig.Disease models & mechanisms · 2026Article
- Single-nucleus transcriptomics of an engineered pig model reveals microglia-T cell interactions driving Huntington's disease neurodegeneration.Nature biomedical engineering · 2026Article
- Factors Influencing the Production Efficiency of Cloned Pigs: A Large-Scale Retrospective Analysis.Animals : an open access journal from MDPI · 2026Article
- Development of a BM7G(TKO/hCD46/hCD55/hTHBD/hEPCR) donor pig with endogenous promoter-driven transgenes for xenotransplantation.Frontiers in immunology · 2026Article
- Evolutionary insights and structural characterization guide the development of RAG1/RAG2-deficient swine models for immunological research.Frontiers in immunology · 2026Article
- Exploring huntington's disease from a neurodevelopmental perspective.International journal of biological sciences · 2026Review
- An integrative neuropharmacological review of Huntington's disease challenges and the role of novel formulations in addressing pharmacological‒pharmaceutical limitations.Frontiers in pharmacology · 2026Review
- Cross-sectional MRI-based quantification of central nervous system tissue and cerebrospinal fluid volumes in juvenile domestic pigs.Frontiers in veterinary science · 2026Article
- Natural products proposed for the management of Huntington's disease (HD): a comprehensive review.Naunyn-Schmiedeberg's archives of pharmacology · 2025Review
123 more citing papers are in PubMed but not listed here.
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Authors and funding
21 authors at 7 institutions in 2 countries.
Sen YanMinistry of Education CNS Regeneration Collaborative Joint Laboratory, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, 510632 Guangzhou, China.
Zhuchi TuMinistry of Education CNS Regeneration Collaborative Joint Laboratory, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, 510632 Guangzhou, China.
Zhaoming LiuKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Nana FanKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Huiming YangDepartment of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.
Su YangDepartment of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.
Weili YangMinistry of Education CNS Regeneration Collaborative Joint Laboratory, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, 510632 Guangzhou, China.
Yu ZhaoKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Zhen OuyangKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Chengdan LaiKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Huaqiang YangKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Li LiKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Qishuai LiuKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Hui ShiKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China.
Guangqing XuDepartment of Neurology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, 510080 Guangdong, China.
Heng ZhaoCollege of Animal Science and Technology, Yunnan Agricultural University, 650201 Kunming, China.
Hongjiang WeiCollege of Animal Science and Technology, Yunnan Agricultural University, 650201 Kunming, China.
Zhong PeiDepartment of Neurology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, 510080 Guangdong, China.
Shihua LiMinistry of Education CNS Regeneration Collaborative Joint Laboratory, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, 510632 Guangzhou, China; Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA. Electronic address: sli@emory.edu.
Liangxue LaiKey Laboratory of Regenerative Biology, South China Institute for Stem Cell, Biology and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, 510530 Guangzhou, China; Jilin Provincial Key Laboratory of Animal Embryo Engineering, Institute of Zoonosis, College of Veterinary Medicine, Jilin University, 130062 Changchun, China. Electronic address: lai_liangxue@gibh.ac.cn.
Xiao-Jiang LiMinistry of Education CNS Regeneration Collaborative Joint Laboratory, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, 510632 Guangzhou, China; Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA. Electronic address: xli2@emory.edu.
Chinese Academy of Sciences · CNJinan University · CNGuangzhou Institutes of Biomedicine and Health · CNYunnan Agricultural University · CNEmory University · USSun Yat-sen University · CNThe First Affiliated Hospital, Sun Yat-sen University · CN
Funding
Neuronal function of huntingtin-associated proteinR01NS036232 · NINDS · EMORY UNIVERSITY · PI ESCAYG, ANDREW P · 1998 to 2021
$6.7MNuclear Toxicity of Huntington Disease ProteinR01NS041669 · NINDS · EMORY UNIVERSITY · PI LI, XIAO-JIANG · 2001 to 2015
$4.6MSynaptic toxicity of Huntington disease proteinR01AG019206 · NIA · EMORY UNIVERSITY · PI LI, XIAO-JIANG · 2001 to 2016
$4.5MPolyglutamine expansion and neuropathologyR01NS045016 · NINDS · EMORY UNIVERSITY · PI LI, SHI-HUA · 2004 to 2013
$2.9MN-terminal huntingtin and Huntington disease neuropathologyR01NS101701 · NINDS · EMORY UNIVERSITY · PI ESCAYG, ANDREW P · 2017 to 2021
$2.2MGlial huntingtin and neurodegenerationR01NS095181 · NINDS · EMORY UNIVERSITY · PI WEINSHENKER, DAVID · 2015 to 2019
$1.7MPolyglutamine Expansion Length Dependent PathologyR01NS095279 · NINDS · EMORY UNIVERSITY · PI WARREN, STEPHEN T. · 2015 to 2019
$1.7MGlial huntingtin and neuronal excitotoxicityR01AG031153 · NIA · EMORY UNIVERSITY · PI LI, SHI-HUA · 2009 to 2013
$1.5MNIA NIH HHS R01 AG019206NIA NIH HHS R01 AG031153NINDS NIH HHS R01 NS036232NINDS NIH HHS R01 NS041669NINDS NIH HHS R01 NS045016NINDS NIH HHS R01 NS095181NINDS NIH HHS R01 NS095279NINDS NIH HHS R01 NS101701
Abstract
Huntington's disease (HD) is characterized by preferential loss of the medium spiny neurons in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we established a knockin (KI) pig model of HD that endogenously expresses full-length mutant huntingtin (HTT). By breeding this HD pig model, we have successfully obtained F1 and F2 generation KI pigs. Characterization of founder and F1 KI pigs shows consistent movement, behavioral abnormalities, and early death, which are germline transmittable. More importantly, brains of HD KI pig display striking and selective degeneration of striatal medium spiny neurons. Thus, using a large animal model of HD, we demonstrate for the first time that overt and selective neurodegeneration seen in HD patients can be recapitulated by endogenously expressed mutant proteins in large mammals, a finding that also underscores the importance of using large mammals to investigate the pathogenesis of neurodegenerative diseases and their therapeutics.
Indexed as
AnimalsBody WeightBrainCerebral CortexCorpus StriatumCRISPR-Cas SystemsDisease Models, AnimalHuntingtin ProteinHuntington DiseaseMagnetic Resonance ImagingNeuronsNuclear Transfer TechniquesSurvival RateSwineTrinucleotide RepeatsHuntingtin ProteinCRISPR/Cas9huntingtinknockinlarge animalneurodegenerationpolyglutaminestriatum
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.