As a leader in neurobiology research, JAX is a resource for you regardless of where you are in your drug discovery pipeline. Not only do we offer the largest collection of neurological mutant mouse models, we offer a wide variety of pre-clinical services for many common mouse models of neurological disorders.
Neuromuscular diseases affect nerves that control voluntary muscles and the nerves that communicate sensory information back to the brain. They are usually genetic in origin and often are characterized by muscle weakness.
Efficacy studies performed by JAX® In Vivo Services evaluate muscle function with readouts such as rotarod performance, time to exhaustion with forced exercise, grip strength, locomotor testing in response to therapeutics, electrophysiological assessment of motor neuropathy, and histological assessment of neuromuscular junctions. We also offer inflammation and fibrosis assessment to determine amelioration following treatment.
See Efficacy Studies for Muscular Dystrophy
Explore Preclinical Solutions for other Neuromuscular Diseases
|STRAIN No.||SIGNIFICANT MODELING|
|These mice express a G93A mutant forms of human SOD1 and may be useful in studying Amyotrophic Lateral Sclerosis.|
|These mice have a transgene encoding human C9Orf72 wit ha hexanucleotide repeat expansion and may be useful is studying ALS.|
|These mice express a mutant human TAR DNA binding protein cDNA harboring an amino acid substitution associated with familial ALS.|
|Also known as mdx, these spontaneous Dmdmdx mutant mice do not express dystrophin and may be useful for studying Duchenne muscular dystrophy.|
|These mice exhibit a molecular and progressive neurodegenerative phenotype similar to Type III spinal muscular atrophy.|
Neurodegenerative disease refers to progressive damage to the central & peripheral nervous system, including the loss of structure and function of neurons.
Efficacy studies performed by JAX® In Vivo Services for neurodegenerative diseases typically feature clinical observations, body weights, progressive neurological scoring, electrophysiological assessment of motor neuropathy, molecular analysis of protein expression levels (including ELISA or western blotting), survival, tissue/blood collection, echocardiography (ECG), and histological assessment of neuromuscular junctions and femoral nerves.
See Efficacy Studies for Friedreich's Ataxia
Explore Preclinical Solutions for other Neurodegenerative Diseases
|STRAIN No.||SIGNIFICANT MODELING|
|Also known as 5XFAD, these mice recapitulate the major features of Alzheimer's Disease amyloid pathology and may be a useful model of intraneuronal Abeta-42 induced neurodegeneration and amyloid plaque formation.|
|Also known as htau, this strain of micelack endogenous Mapt gene expression, and express all six isoforms of human MAPT. This model may be useful in studying Alzheimer’s Disease pathogenesis.|
|These mice may be useful in studying human neuronal alpha-synucleinopathies, such as familial Parkinson's Disease.|
|These transgenic mice display a progressive neurological phenotype that mimics many of the features of Huntington Disease (HD) in humans.|
B6.Cg-Pvalbtm1(cre)Arbr Fxnem2Lutzy Fxnem2.1Lutzy/J
|This FRDA model develops ataxia due to frataxin protein deficiency in parvalbumin-expressing neurons, and maybe useful in studying Friedreich’s Ataxia.|
Neurodevelopmental diseases are those that affect neurological development due to a genetic origin (e.g., Down Syndrome, Fragile X Syndrome, Rett Syndrome) or a multifactorial origin such as the environment, that can impair some aspect of neurodevelopment (e.g., ADHD, autism, Asperger’s, sensory processing disorders).
Efficacy studies in Rett Syndrome performed by JAX® In Vivo Services for neurodevelopmental disorders primarily utilize whole body plethysmography – measurement of breathing irregularity (also known as “breath holds”) and seizure activity. Other offerings for neurodevelopmental disorders include neurological scoring and locomotor assessment in response to therapeutics, associated data analyses, and tissue and blood/plasma collection.
|STRAIN No.||SIGNIFICANT MODELING|
|Ts65Dn mice are a model for studying Down Syndrome, and are trisomic for about two-thirds of the genes orthologous to human chromosome 21.
Explore the cytogenetic and down syndrome resources.
|Also known as Mecp2-, this strain is a model of Rett Syndrome. Male mice exhibit mobility problem and a range of Rett Syndrome-like characteristics.|
|Also known as CDkl5, this model exhibits Autistic-like behavioral abnormalities, deficits in neural circuit communication, and alterations in multiple signal transduction pathways. This model can be useful in studying disorders such as Rett syndrome, autism spectrum disorders, and early infantile epileptic encephalopathy.|
|Defects in FMR1 cause Fragile X Syndrome; these mice show phenotypes consistent with the deficits seen in common forms of this disease.|
FVB.129P2-Pde6b+ Tyrc-ch Fmr1tm1Cgr/J
|This model of Fragile X Syndrome exhibits behavioral deficits consistent with the disease, as well no blindness due to unrelated retinal degeneration.|
As a global leader in mammalian genetics and preclinical disease modeling, our passion is empowering the development of novel therapeutics in our shared quest to advance human health. Our preclinical services offer robust scientific expertise, numerous in life and end point measurements, phenotyping capabilities, and customizable study design for the therapeutic evaluation of many neurological disorder models.
Reach out to us to learn more about our in-depth repository of neurological models or explore how we can work together to generate a custom, pre-clinical solution for your therapeutic development goals.
In partnership with The Chan Zuckerberg Initiative and NIH, The Jackson Laboratory offers a collection of genetically engineered iPS cell lines carrying single nucleotide variants (SNVs) relevant to neurodegenerative disease. Paired with an array of carefully crafted genetic controls, these iPS cell lines offer an efficient and reliable tool set for investigating potential biomarkers and testing therapeutic strategies.
JAX® Mice are the industry standard for animal model research, using precise genome solutions to better understand human disease.Learn More
Over 30,000 peer-reviewed publications cite the use of JAX® Mice strains. Hundreds of new papers referencing JAX® Mice are published each month. To complement this published information, we routinely conduct studies to gather physiological data on JAX® Mice models.Learn More
Humanized NSG™ and NSG™-SGM3 mouse models are powerful tools for studying cancer, inflammatory and infectious disease, and hematopoiesis. We maintain large on-the-shelf inventories of two versatile humanized models. Both are popular models for checkpoint inhibitor development.Learn More
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