Amyotrophic Lateral Sclerosis ALS models

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease characterized by increasing weakness and muscle wasting. ALS is also known as motor neurone disease (MND), Lou Gehrig's disease, and Charcot disease.

Mouse models of ALS mimic different aspects of the disease, such as toxic aggregation of ALS-related proteins and loss of motor neurons. We have also detected significant motor problems in our tests of motor function.

Transgenic SOD1 models

Mutations in the superoxide dismutase 1 (SOD1) gene are associated with familial ALS. Mice that express the human protein with ALS-related mutation have significant SOD1 aggregation, oxidative stress, and motor neuron loss. Sylics has detected significant motor problems in transgenic SOD1 mice in our test of motor function (e.g. Activity in an automated home-cage, Grip strength, Rotarod). The behavioral changes can be detected already at young ages in SOD1 mice and are progressive, providing a large time window for testing new therapeutics.

SOD1 mutant mice show progressive behavioral changes in the PhenoTyper including a reduced frequency to climb on top of their shelter.
Motor impairment in VWM mice (Beam Test)

Eif2b mutant mice

Inhibition of eukaryotic initiation factor EIF2b-mediated protein synthesis during cellular stress is thought to play a key role in ALS. Activators of EIF2b are therefore explored clinically for the potential treatment of ALS. Mutations in Eif2b lead to a childhood disease called vanishing white matter (VWM). Mice carrying these human mutations recapitulate many of the phenotypes of VWM and are therefore an attractive model for testing Eif2b-related drugs for ALS. Sylics offers unique services with an Eif2b4 / Eif2b5 mouse model through a collaboration with the Amsterdam Leukodystrophy Center at Amsterdam UMC. See this vanishing white matter for more information.

Transgenic TDP43 models

Mutations in the gene encoding TAR DNA-binding protein 43 (TDP-43) are associated with familial ALS and virtually all sporadic ALS cases have TDP-43 inclusions.Pathological TDP43 is therefore thought to play a key role in the disease pathogenesis. Sylics offers free-for-service transgenic TDP43 mouse models, which recapitulate the TDP43 aggregates found in ALS and frontotemporal dementia (FTD). For more information please contact us.

TDP43 seeding models

TDP-43 pathology progresses through the brain and closely correlates with disease symptoms in ALS. It is increasingly recognized that transneuronal spreading of TDP-43 pathology plays a key role in the pathophysiology of ALS and FTD. Injection of brain extracts from these diseases in the brain of mice closely mimics the structural features of the TDP-43 aggregates found in patients and allows studying of TDP-43 spreading as a therapeutic readout. Sylics has extensive experience with seeding-based mouse models, based on both recombinant and patient-derived seeds. For more information please contact us.

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