NSTL国家科技图书文献中心

1. Humanized rodent models of neurodegenerative diseases and other brain disorders NSTL国家科技图书文献中心

Zhang, Xinru | Wang, Jianxiang ... - 《Neuroscience and Biobehavioral Reviews》 - 2025,172 - Article 106112～Article 106112 - 共18页

摘要： onset, progression, and pathology of these diseases in | studying human CNS diseases and their therapies in vivo | , significance, and limitations of humanized rodent models in | the opportunities for in vivo exploration of human | Central Nervous System (CNS) diseases

关键词： Humanized model | Central nervous system diseases | Transgenic | Humanized chimeric mouse | Gene knock-in model | Pathogenesis

2. RNA Interference Targeting Small Heat Shock Protein B8 Failed to Improve Distal Hereditary Motor Neuropathy in the Mouse Model NSTL国家科技图书文献中心

Vendredy, Leen | De Winter, Vicky ... - 《The journal of gene medicine》 - 2025,27(2) - n/a～n/a - 共18页

摘要： pluripotent stem cells and the Hspb8 knock-in mouse model | BackgroundMissense mutations in the HSPB8 gene | phenotype in patient-derived motor neurons and in a knock | -in mouse model* of CMT2L/dHMN.MethodsWe optimized* | knock-in mice showed inconclusive results towards

关键词： adeno-associated virus | Charcot-Marie-Tooth neuropathy | magnetic resonance imaging | RNA interference | small heat shock protein | adeno‐associated virus | Charcot–Marie–Tooth neuropathy

3. Effectiveness of a Novel PLA2R1 Knock‐In Rat Model in Repairing Renal Function Damage NSTL国家科技图书文献中心

Bo Huang | Wen‐dong Sui ... - 《Journal of biochemical and molecular toxicology》 - 2025,39(1) - e70056～e70056 - 共11页

摘要： a PLA2R1 knock‐in rat model* with repaired kidney* | (−)] model* and PLA2R1 knock in [PLA2R1(+)] model in rats* | PLA2R1(+) model* than in the PLA2R1(−) model, and some* | ) exists in many animals and plays an important role in | membranous nephropathy. In this study, we aimed to evaluate

关键词： complement deposition | knock‐in | membranous nephropathy | phospholipase A2 receptor 1 | rat model

4. The L348P point mutation in cardiac myosin binding protein-C alters transient responses to stretch, slows cardiac relaxation, and is embryonic lethal in homozygous CRISPR gene-edited mice NSTL国家科技图书文献中心

Sadler, Rachel L. | Greenman, Angela C.... - 《Journal of Molecular and Cellular Cardiology》 - 2025,203 - 35～46 - 共12页

摘要： L348P-CR knock-in mice are a robust model* of diastolic* | mutant protein by creating a CRISPR gene-edited knock | -in mouse model* (L348P-CR) and breeding mice to* | heterozygous knock-in mice developed contractile deficits and | Mutations in cardiac myosin binding protein-C

关键词： Cardiac myosin binding protein-C | Diastolic dysfunction | SpyCatcher | SpyTag | Cardiomyocyte

5. AML1-ETO and CCND2 overexpression cooperate to drive acute myeloid leukemia initiation and progression NSTL国家科技图书文献中心

Mou, Junli | Huang, Qianqian ... - 《Journal of Leukocyte Biology》 - 2025,117(6) - qiaf072～qiaf072 - 共9页

摘要：-in mouse model* (AML1-ETO mouse), which represented* | detected CCND2 mutations in acute myeloid leukemia | , especially in the subtype of acute myeloid leukemia with | AML1-ETO fusion gene. However, the AML1-ETO fusion | gene alone is not sufficient to drive leukemia

关键词： AML-ETO | CCND2 | everolimus | mTOR | second hit

6. Evaluation of Physiological Integrity in Six-Gene-Edited Bama Miniature Pigs as a Model for Xenotransplantation NSTL国家科技图书文献中心

Wen Chuan Peng | Yuan Yuan Zhai ... - 《Biotechnology Journal》 - 2025,20(5) - e70030～17 - 共17页

摘要： immune system. In this study, we generated a six-gene | with human transgene knock-in BD and complement genes | significant potential as a solution to organ scarcity in | xenotransplantation. Nonetheless, a formidable challenge lies in | -edited pig model* by simultaneously knocking out three*

关键词： genetic engineering | multiplex CRISPR/Cas9 gene editing | six-gene-edited pig | xenotransplantation

7. G3BP1, a stress granule core protein, ameliorates metabolic dysfunction-associated fatty liver disease by attenuating hepatocyte lipid deposition NSTL国家科技图书文献中心

Liu, Xingjing | Yu, Huimei ... - 《Diabetes, obesity & metabolism.》 - 2025,27(6) - 2985～2995 - 共11页

摘要： stress granules (SGs) in MAFLD.MethodsA gene* knock-down* | model of G3BP1, a core SG molecule in mice and HepG2 | cells, was constructed to explore the role of SGs in | MAFLD induced in vivo by a high-fat diet or in vitro | ).ResultsG3BP1 and TIA1 expression were upregulated in high-fat

关键词： APOC3 | fatty acid | G3BP1 | Metabolic dysfunction-associated fatty liver disease | stress granule | Metabolic dysfunction‐associated fatty liver disease

8. Construction of the Gene Tagging and Knock Out system for reliable genetic analysis of nuclear genes in Cyanidioschyzon merolae NSTL国家科技图书文献中心

Bora, Prerna | Tanaka, Kan - 《Plant and cell physiology》 - 2025,66(3) - 374～383 - 共10页 - 被引量：1

摘要： mutant design system Gene* Tagging and Knock Out (GTKO* | merolae is a eukaryotic photosynthetic model* organism* | unstable phenotypes. In this study, we modified the pMKT | vector system described in Takemura et al. (Takemura, T | use in epitope tagging of multiple nuclear genes in

关键词： Cyanidioschyzon merolae | gene knockout | isogenic control | phenotypic analysis

9. Generation of a novel constitutive smooth muscle cell-specific Myh11 -driven Cre mouse model NSTL国家科技图书文献中心

Dong, Kunzhe | Bai, Zhixia ... - 《Journal of Molecular and Cellular Cardiology》 - 2025,202 - 144～152 - 共9页

摘要： mouse model* generated by knock-in (KI) of a nuclear* | gene, particularly in adult mice. For studies | gene function specifically in SMCs during embryonic | Dysfunction in either embryonic or postnatal | crucial. MYH11 is the most reliable lineage gene* for*

关键词： Smooth muscle | Myosin heavy chain | Myh11 | Cre | Knock-in | Embryonic development | Transcription factor | Tead1

10. A study into rare GPR146 gene variants in humans and mice NSTL国家科技图书文献中心

Zhang, Boyan | Rimbert, Antoine ... - 《Atherosclerosis》 - 2025,403 - Article 119137～Article 119137 - 共9页

摘要： Consortium (GLGC) data set and in a knock-in mouse model | coding variants in GPR146. We first carried out gene | 21% reduction in plasma cholesterol. This is | reduced SREBP2 activity in the liver, which leads to | in humans is however limited. We therefore set out

摘要： onset, progression, and pathology of these diseases in | studying human CNS diseases and their therapies in vivo | , significance, and limitations of humanized rodent models in | the opportunities for in vivo exploration of human | Central Nervous System (CNS) diseases

关键词： Humanized model | Central nervous system diseases | Transgenic | Humanized chimeric mouse | Gene knock-in model | Pathogenesis

摘要： pluripotent stem cells and the Hspb8 knock-in mouse model | BackgroundMissense mutations in the HSPB8 gene | phenotype in patient-derived motor neurons and in a knock | -in mouse model* of CMT2L/dHMN.MethodsWe optimized* | knock-in mice showed inconclusive results towards

关键词： adeno-associated virus | Charcot-Marie-Tooth neuropathy | magnetic resonance imaging | RNA interference | small heat shock protein | adeno‐associated virus | Charcot–Marie–Tooth neuropathy

关键词： complement deposition | knock‐in | membranous nephropathy | phospholipase A2 receptor 1 | rat model

摘要： L348P-CR knock-in mice are a robust model* of diastolic* | mutant protein by creating a CRISPR gene-edited knock | -in mouse model* (L348P-CR) and breeding mice to* | heterozygous knock-in mice developed contractile deficits and | Mutations in cardiac myosin binding protein-C

关键词： Cardiac myosin binding protein-C | Diastolic dysfunction | SpyCatcher | SpyTag | Cardiomyocyte

摘要：-in mouse model* (AML1-ETO mouse), which represented* | detected CCND2 mutations in acute myeloid leukemia | , especially in the subtype of acute myeloid leukemia with | AML1-ETO fusion gene. However, the AML1-ETO fusion | gene alone is not sufficient to drive leukemia

关键词： AML-ETO | CCND2 | everolimus | mTOR | second hit

关键词： genetic engineering | multiplex CRISPR/Cas9 gene editing | six-gene-edited pig | xenotransplantation

摘要： stress granules (SGs) in MAFLD.MethodsA gene* knock-down* | model of G3BP1, a core SG molecule in mice and HepG2 | cells, was constructed to explore the role of SGs in | MAFLD induced in vivo by a high-fat diet or in vitro | ).ResultsG3BP1 and TIA1 expression were upregulated in high-fat

关键词： APOC3 | fatty acid | G3BP1 | Metabolic dysfunction-associated fatty liver disease | stress granule | Metabolic dysfunction‐associated fatty liver disease

摘要： mutant design system Gene* Tagging and Knock Out (GTKO* | merolae is a eukaryotic photosynthetic model* organism* | unstable phenotypes. In this study, we modified the pMKT | vector system described in Takemura et al. (Takemura, T | use in epitope tagging of multiple nuclear genes in

关键词： Cyanidioschyzon merolae | gene knockout | isogenic control | phenotypic analysis

摘要： mouse model* generated by knock-in (KI) of a nuclear* | gene, particularly in adult mice. For studies | gene function specifically in SMCs during embryonic | Dysfunction in either embryonic or postnatal | crucial. MYH11 is the most reliable lineage gene* for*

关键词： Smooth muscle | Myosin heavy chain | Myh11 | Cre | Knock-in | Embryonic development | Transcription factor | Tead1

摘要： Consortium (GLGC) data set and in a knock-in mouse model | coding variants in GPR146. We first carried out gene | 21% reduction in plasma cholesterol. This is | reduced SREBP2 activity in the liver, which leads to | in humans is however limited. We therefore set out

关键词： Rare variant | Cholesterol | Mutation | GPR146

4008-161-200 800-990-8900

国家科技图书文献中心

摘要： onset, progression, and pathology of these diseases in | studying human CNS diseases and their therapies in vivo | , significance, and limitations of humanized rodent models in | the opportunities for in vivo exploration of human | Central Nervous System (CNS) diseases

关键词： Humanized model | Central nervous system diseases | Transgenic | Humanized chimeric mouse | Gene knock-in model | Pathogenesis

摘要： pluripotent stem cells and the Hspb8 knock-in mouse model | BackgroundMissense mutations in the HSPB8 gene | phenotype in patient-derived motor neurons and in a knock | -in mouse model of CMT2L/dHMN.MethodsWe optimized | knock-in mice showed inconclusive results towards

关键词： adeno-associated virus | Charcot-Marie-Tooth neuropathy | magnetic resonance imaging | RNA interference | small heat shock protein | adeno‐associated virus | Charcot–Marie–Tooth neuropathy

摘要： a PLA2R1 knock‐in rat model with repaired kidney | (−)] model and PLA2R1 knock in [PLA2R1(+)] model in rats | PLA2R1(+) model than in the PLA2R1(−) model, and some | ) exists in many animals and plays an important role in | membranous nephropathy. In this study, we aimed to evaluate

关键词： complement deposition | knock‐in | membranous nephropathy | phospholipase A2 receptor 1 | rat model

摘要： L348P-CR knock-in mice are a robust model of diastolic | mutant protein by creating a CRISPR gene-edited knock | -in mouse model (L348P-CR) and breeding mice to | heterozygous knock-in mice developed contractile deficits and | Mutations in cardiac myosin binding protein-C

关键词： Cardiac myosin binding protein-C | Diastolic dysfunction | SpyCatcher | SpyTag | Cardiomyocyte

摘要：-in mouse model (AML1-ETO mouse), which represented | detected CCND2 mutations in acute myeloid leukemia | , especially in the subtype of acute myeloid leukemia with | AML1-ETO fusion gene. However, the AML1-ETO fusion | gene alone is not sufficient to drive leukemia

关键词： AML-ETO | CCND2 | everolimus | mTOR | second hit

关键词： genetic engineering | multiplex CRISPR/Cas9 gene editing | six-gene-edited pig | xenotransplantation

摘要： stress granules (SGs) in MAFLD.MethodsA gene knock-down | model of G3BP1, a core SG molecule in mice and HepG2 | cells, was constructed to explore the role of SGs in | MAFLD induced in vivo by a high-fat diet or in vitro | ).ResultsG3BP1 and TIA1 expression were upregulated in high-fat

关键词： APOC3 | fatty acid | G3BP1 | Metabolic dysfunction-associated fatty liver disease | stress granule | Metabolic dysfunction‐associated fatty liver disease

摘要： mutant design system Gene Tagging and Knock Out (GTKO | merolae is a eukaryotic photosynthetic model organism | unstable phenotypes. In this study, we modified the pMKT | vector system described in Takemura et al. (Takemura, T | use in epitope tagging of multiple nuclear genes in

关键词： Cyanidioschyzon merolae | gene knockout | isogenic control | phenotypic analysis

摘要： mouse model generated by knock-in (KI) of a nuclear | gene, particularly in adult mice. For studies | gene function specifically in SMCs during embryonic | Dysfunction in either embryonic or postnatal | crucial. MYH11 is the most reliable lineage gene for

关键词： Smooth muscle | Myosin heavy chain | Myh11 | Cre | Knock-in | Embryonic development | Transcription factor | Tead1

摘要： Consortium (GLGC) data set and in a knock-in mouse model | coding variants in GPR146. We first carried out gene | 21% reduction in plasma cholesterol. This is | reduced SREBP2 activity in the liver, which leads to | in humans is however limited. We therefore set out

关键词： Rare variant | Cholesterol | Mutation | GPR146

4008-161-200 800-990-8900

国家科技图书文献中心

摘要： pluripotent stem cells and the Hspb8 knock-in mouse model | BackgroundMissense mutations in the HSPB8 gene | phenotype in patient-derived motor neurons and in a knock | -in mouse model* of CMT2L/dHMN.MethodsWe optimized* | knock-in mice showed inconclusive results towards

摘要： L348P-CR knock-in mice are a robust model* of diastolic* | mutant protein by creating a CRISPR gene-edited knock | -in mouse model* (L348P-CR) and breeding mice to* | heterozygous knock-in mice developed contractile deficits and | Mutations in cardiac myosin binding protein-C

摘要：-in mouse model* (AML1-ETO mouse), which represented* | detected CCND2 mutations in acute myeloid leukemia | , especially in the subtype of acute myeloid leukemia with | AML1-ETO fusion gene. However, the AML1-ETO fusion | gene alone is not sufficient to drive leukemia

摘要： stress granules (SGs) in MAFLD.MethodsA gene* knock-down* | model of G3BP1, a core SG molecule in mice and HepG2 | cells, was constructed to explore the role of SGs in | MAFLD induced in vivo by a high-fat diet or in vitro | ).ResultsG3BP1 and TIA1 expression were upregulated in high-fat

摘要： mutant design system Gene* Tagging and Knock Out (GTKO* | merolae is a eukaryotic photosynthetic model* organism* | unstable phenotypes. In this study, we modified the pMKT | vector system described in Takemura et al. (Takemura, T | use in epitope tagging of multiple nuclear genes in

摘要： mouse model* generated by knock-in (KI) of a nuclear* | gene, particularly in adult mice. For studies | gene function specifically in SMCs during embryonic | Dysfunction in either embryonic or postnatal | crucial. MYH11 is the most reliable lineage gene* for*