Mouse Models in the Study of Genetic Neurological Disorders

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Humans have been breeding animals for millenia to bring out desirable characteristics. With the thoroughbred race horse there's lots of money at stake but with research mice it's the possibility of life-saving new treatments. Can you spare minutes to tell us what you think of this website? Open survey. In: Facts Animals and Plants.

Key facts Over the past century, the house mouse Mus musculus has become the preferred mammalian model for genetic research. In the early days of biomedical research, scientists developed mouse models by selecting and breeding specific mice to produce offspring with certain desired characteristics.

Now scientists use mice to simulate human genetic disorders in order to study their development and test new therapies. As a scientific tool, mice have helped to speed up the progress of research and enabled the development of important new drugs.

Mouse genes could help decipher human disease

The genome sequence of the mouse was published in December Its genome is approximately 3, million base pairs in length and contains over 23, protein-coding genes Ensembl. An adult black mouse. Image credit: Wellcome Library, London. Related Content:. Of mice and men. What are model organisms? Nat Genet. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol. Myers R. Huntington's disease genetics. The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis.

Mov Disord. Role of tetrabenazine for Huntington's disease-associated chorea. Ann Pharmacother. Clinical assessment of the effect of tetrabenazine on functional scales in huntington disease: a pilot open label study. The long-term effect of tetrabenazine in the management of Huntington disease. Clin Neuropharmacol. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.

Mouse models of polyglutamine diseases in therapeutic approaches: review and data table.

Transgenic Mouse Models and Human Neurodegenerative Disorders

Part II. Mol Neurobiol. Sci Rep. The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies. Ferrante R. Mouse models of Huntington's disease and methodological considerations for therapeutic trials. Biochim Biophys Acta. Behav Brain Res. Selective neurodegeneration, neuropathology and symptom profiles in Huntington's disease.

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Adv Exp Med Biol. Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease. J Biol Chem. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J Neurosci. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. A fully humanized transgenic mouse model of Huntington disease. Hum Mol Genet. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage.

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

Mouse Models in the Study of Genetic Neurological Disorders

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. PLoS Curr. Subtle but progressive cognitive deficits in the female tgHD hemizygote rat as demonstrated by operant SILT performance.

Brain Res Bull. Transgenic rat model of Huntington's disease. Profiles of motor and cognitive impairment in the transgenic rat model of Huntington's disease. Adams C, Brantner V. Spending on new drug development1. Health Econ. Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model.

Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Characterization of the Huntington's disease HD gene homologue in the zebrafish Danio rerio.

Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation. A genomewide RNA interference screen for modifiers of aggregates formation by mutant Huntingtin in Drosophila. Identification of anti-prion compounds as efficient inhibitors of polyglutamine protein aggregation in a zebrafish model.

Giacomotto J, Segalat L. High-throughput screening and small animal models, where are we?. Br J Pharmacol.

Frontiers | Mouse Genetic Models of Human Brain Disorders | Genetics

Modeling neurodegeneration in zebrafish. Curr Neurol Neurosci Rep. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Novel targets for Huntington's disease in an mTOR-independent autophagy pathway. Nat Chem Biol. Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.

Mol Ther. Towards a transgenic model of Huntington's disease in a non-human primate. Morton A, Avanzo L. Executive decision-making in the domestic sheep. Gagliardi C, Bunnell B. Large animal models of neurological disorders for gene therapy. ILAR J. Neuroprotective gene therapy for Huntington's disease, using polymer-encapsulated cells engineered to secrete human ciliary neurotrophic factor: results of a phase I study. Hum Gene Ther. Behavioral and morphological comparison of two nonhuman primate models of Huntington's disease.

A primate model of Huntington's disease: behavioral and anatomical studies of unilateral excitotoxic lesions of the caudate-putamen in the baboon. Exp Neurol. Excitotoxin lesions in primates as a model for Huntington's disease: histopathologic and neurochemical characterization. Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy. Drug Des Devel Ther.

An ovine transgenic Huntington's disease model. Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease. Nat Med. Lew M. Overview of Parkinson's disease. Parkinson's disease. Dorsey E, Bloem B. JAMA Neurol. Parkinson's disease and pesticides: a toxicological perspective. Trends Pharmacol Sci.

About the Author

Migliore L, Coppede F. Environmental-induced oxidative stress in neurodegenerative disorders and aging. Mutat Res. Genetics, environmental factors and the emerging role of epigenetics in neurodegenerative diseases.

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    Review ARTICLE

    The 6-hydroxydopamine model of Parkinson's disease. Neurotox Res. Nucl Med Biol. Effects of unilateral 6-hydroxydopamine lesions on neuropeptide immunoreactivity in the basal ganglia of the common marmoset, Callithrix jacchus, a quantitative immunohistochemical analysis. J Chem Neuroanat. Evaluation of simple and complex sensorimotor behaviours in rats with a partial lesion of the dopaminergic nigrostriatal system. Eur J Neurosci. Characterization of behavioral and neurodegenerative changes following partial lesions of the nigrostriatal dopamine system induced by intrastriatal 6-hydroxydopamine in the rat.

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    Sonsalla P, Heikkila R. The influence of dose and dosing interval on MPTP-induced dopaminergic neurotoxicity in mice. Eur J Pharmacol. The impact of gender and estrogen on striatal dopaminergic neurotoxicity. Ann N Y Acad Sci. Cold Spring Harb Perspect Med.

    Mouse Models in the Study of Genetic Neurological Disorders Mouse Models in the Study of Genetic Neurological Disorders
    Mouse Models in the Study of Genetic Neurological Disorders Mouse Models in the Study of Genetic Neurological Disorders
    Mouse Models in the Study of Genetic Neurological Disorders Mouse Models in the Study of Genetic Neurological Disorders
    Mouse Models in the Study of Genetic Neurological Disorders Mouse Models in the Study of Genetic Neurological Disorders
    Mouse Models in the Study of Genetic Neurological Disorders Mouse Models in the Study of Genetic Neurological Disorders

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