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  1. Methyl β-Cyclodextrin-sperm-mediated gene editing (MBCD-SMGE): a simple and efficient method for targeted mutant mouse production

    Background

    Generating targeted mutant mice is a crucial technology in biomedical research. This study focuses on optimizing the CRISPR/Cas9 system...

    Parisa Moradbeigi, Sara Hosseini, ... Asghar Mogheiseh in Biological Procedures Online
    Article Open access 26 January 2024
  2. Lurcher Mouse

    Lurcher mutant mouse is a natural model of hereditary cerebellar degeneration which is caused by a mutation in the δ2 glutamate receptor encoding...
    Jan Cendelin, Jan Tuma, Zdenka Purkartova in Essentials of Cerebellum and Cerebellar Disorders
    Chapter 2023
  3. The Rolling Nagoya Mouse

    The natural mutant mouse rolling Nagoya is severely ataxic and frequently shows body roll-overs. The phenotype is inherited in an autosomal recessive...
    Jaap J. Plomp, Arn M. J. M. van den Maagdenberg, Else A. Tolner in Essentials of Cerebellum and Cerebellar Disorders
    Chapter 2023
  4. Long-term inhibition of mutant LRRK2 hyper-kinase activity reduced mouse brain α-synuclein oligomers without adverse effects

    Parkinson’s disease (PD) is characterized by dopaminergic neurodegeneration in nigrostriatal and cortical brain regions associated with pathogenic...

    Philip Wing-Lok Ho, Eunice Eun-Seo Chang, ... Shu-Leong Ho in npj Parkinson's Disease
    Article Open access 10 September 2022
  5. Moonwalker Mouse

    The Moonwalker (Mwk) mouse is a dominant ataxic mouse model of inherited cerebellar ataxia caused by a gain-of-function mutation in the gene encoding...
    Mohamed F. Ibrahim, Esther B. E. Becker in Essentials of Cerebellum and Cerebellar Disorders
    Chapter 2023
  6. Rolling Nagoya Mouse

    The natural mutant mouse rolling Nagoya has an uncoordinated gait and frequently displays sideways body rolls. The mutation underlying this autosomal...
    Else A. Tolner, Arn M. J. M. van den Maagdenberg, Jaap J. Plomp in Handbook of the Cerebellum and Cerebellar Disorders
    Reference work entry 2022
  7. LRRK2 mutant knock-in mouse models: therapeutic relevance in Parkinson's disease

    Mutations in the leucine-rich repeat kinase 2 gene ( LRRK2 ) are one of the most frequent genetic causes of both familial and sporadic Parkinson’s...

    Eunice Eun Seo Chang, Philip Wing-Lok Ho, ... Shu-Leong Ho in Translational Neurodegeneration
    Article Open access 14 February 2022
  8. The Staggerer Mouse: RORα Deficiency Induces Cerebellar Neurodegeneration

    The staggerer mutant mouse carries a spontaneous mutation in the ligand-binding domain of the rora gene. RORα is expressed in many tissues and its...
    Natalie Morellini, Ann M. Lohof, ... Rachel M. Sherrard in Essentials of Cerebellum and Cerebellar Disorders
    Chapter 2023
  9. Dual inhibition of SUMOylation and MEK conquers MYC-expressing KRAS-mutant cancers by accumulating DNA damage

    Background

    KRAS mutations frequently occur in cancers, particularly pancreatic ductal adenocarcinoma, colorectal cancer, and non-small cell lung...

    Hiroshi Kotani, Hiroko Oshima, ... Seiji Yano in Journal of Biomedical Science
    Article Open access 11 July 2024
  10. Phenotypic Analysis of Early Neurogenesis in a Mouse Chimeric Embryo and Stem Cell-Based Neuruloid Model

    Analyzing the impact of genetic mutations on early neurogenesis of mammalian embryos in conventional mouse mutant models is laborious and...
    Riley McMahon, V. Pragathi Masamsetti, Patrick P. L. Tam in Neurobiology
    Protocol 2024
  11. Trametinib sensitizes KRAS-mutant lung adenocarcinoma tumors to PD-1/PD-L1 axis blockade via Id1 downregulation

    Background

    The identification of novel therapeutic strategies to overcome resistance to the MEK inhibitor trametinib in mutant KRAS lung...

    Ander Puyalto, María Rodríguez-Remírez, ... Ignacio Gil-Bazo in Molecular Cancer
    Article Open access 20 April 2024
  12. Lurcher Mouse

    Lurcher mutant mice represent one of the frequently used mouse models of the olivocerebellar degenerationOlivocerebellar degeneration. It is caused...
    Jan Cendelin, Frantisek Vozeh in Handbook of the Cerebellum and Cerebellar Disorders
    Reference work entry 2022
  13. Disease-Associated Q159X Mutant Prion Protein Is Sufficient to Cause Fatal Degenerative Disease in Mice

    PRNP Q160X is one of the five dominantly inheritable nonsense mutations causing familial prion diseases. Till now, it remains unclear how this type...

    Yan Zhang, Runchuan Yan, ... Jiyan Ma in Molecular Neurobiology
    Article 14 May 2024
  14. Genetic alterations that deregulate RB and PDGFRA signaling pathways drive tumor progression in IDH2-mutant astrocytoma

    In IDH -mutant astrocytoma, IDH2 mutation is quite rare and biological mechanisms underlying tumor progression in IDH2 -mutant astrocytoma remain...

    Kensuke Tateishi, Yohei Miyake, ... Hiroaki Wakimoto in Acta Neuropathologica Communications
    Article Open access 27 November 2023
  15. Moonwalker Mouse

    The Moonwalker (Mwk) mouse is a model of dominantly inherited cerebellar ataxia. The ataxic phenotype is caused by a gain-of-function mutation in the...
    Reference work entry 2022
  16. Anti-apoptotic Splicing Variant of AIMP2 Recover Mutant SOD1-Induced Neuronal Cell Death

    Although a couple of studies have reported that mutant superoxide dismutase 1 (SOD1), one of the causative genes of familial amyotrophic lateral,...

    Myung Geun Kook, Mi Ran Byun, ... ** Woo Choi in Molecular Neurobiology
    Article 15 October 2022
  17. Microglia Preserve Visual Function in a Mouse Model of Retinitis Pigmentosa with Rhodopsin-P23H Mutant

    Most forms of outer retinal degenerative diseases involve the ectopic accumulation of microglia/macrophages in the subretinal space, including...
    Chen Yu, Daniel R. Saban in Retinal Degenerative Diseases XIX
    Conference paper 2023
  18. Stalled oligodendrocyte differentiation in IDH-mutant gliomas

    Background

    Roughly 50% of adult gliomas harbor isocitrate dehydrogenase ( IDH ) mutations. According to the 2021 WHO classification guideline, these...

    Yanfei Wei, Guanzhang Li, ... **aolong Fan in Genome Medicine
    Article Open access 13 April 2023
  19. A Facile Method to Append a Bio-ID Tag to Endogenous Mutant Kras Alleles

    KRAS mutations occur in approximately ~50% of colorectal cancers (CRCs) and are associated with poor prognosis and resistance to therapy. While these...
    Konstantin Budagyan, Alexa C. Cannon, Jonathan Chernoff in KRAS
    Protocol 2024
  20. Robotic Mouse

    Gene targeting is a very powerful approach for the generation of clinically relevant mouse models to elucidate the underlying molecular basis of...
    Emmanuelle Bitoun, Peter L. Oliver, Kay E. Davies in Handbook of the Cerebellum and Cerebellar Disorders
    Reference work entry 2022
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