Animal models amyotrophic lateral sclerosis

Cork, L. C., Griffin, I. W., Adams, R. J. and Price, D. L. Motor neuron disease spinal muscular atrophy and amyotrophic lateral sclerosis. Animal model hereditary canine spinal muscular atrophy. Am. J. Pathol. 100 599-602,1980. 

Keywords Alzheimer s disease Amyotrophic lateral sclerosis Animal models Apoptosis Huntington s disease Neurodegeneration Neuroprotection Parkinson s disease Randomized controlled trials Therapeutics... 

Alzheimer s disease, Parkinson s disease, Huntington s disease and amyotrophic lateral sclerosis (ALS) are four prominent fatal neurodegenerative disorders that involve the death of specific populations of neurons (see details in respective chapters). Studies of patients and animal and culture models have provided considerable insight in the cellular and molecular mechanisms responsible for synaptic dysfunction and neuronal degeneration in each disorder [18], In Alzheimer s disease, abnormalities in proteolytic processing of the amyloid precursor protein, due to gene... 

It shows significant activity against a spectmm of neurodegenerative disorders in animal models that replicate many of the features of important human neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and Parkinson s disease [147]. 

Canton T, Bohme GA, Boireau A, et al (2001) RPR 119990, a novel alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid antagonist synthesis, pharmacological properties, and activity in an animal model of amyotrophic lateral sclerosis. J Pharmacol Exp Ther 299 314-322... 

Coenzyme Q10 and vitamin E are found in wheat germ. Furthermore, studies in mice have shown that administration of coenzyme Q10 elevate the level of mitochondrial a-tocopherol (Lass et al., 1999). Beal and his colleagues (2002) investigated the potential usefulness of coenzyme Q10 in animal models of PD, amyotrophic lateral sclerosis (ALS), and HD. It has been demonstrated that coenzyme Q10 can protect against striatal lesions produced by the mitochondrial toxins malonate and 3-nitropropionic acid. These toxins have been utilized to model the striatal pathology, which... 

R., Morrison, J. H. and Gordon, J. W. (1995) Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA 92, 689-693. 

Wang, L. J. et al. (2002b). Neuroprotective effects of glial cell line-derived neurotrophic factor mediated by an adeno-associated virus vector in a transgenic animal model of amyotrophic lateral sclerosis. J. Neurosci. 22 (16), 6920-6928. 

Primate Animal Models. . . Amyotrophic Lateral Sclerosis (ALS)... 

Appel SH, Engelhardt JI, Garcia J, Stefani E (1991) Immunoglobulins from animal models of motor neuron disease and from human amyotrophic lateral sclerosis patients passively transfer physiological abnormalities to the neuromuscular junction. Proc Natl Acad SciU S A88 647-651. 

Azzouz M, Leclerc N, Gurney M, Warter JM, Poindron P, et al. 1997. Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis. Muscle Nerve 20 45-51. 

Some NOS inhibitors have been used in research in humans. The nonselective inhibitor NMMA has been used in normals and in trials for septic shock (Bakker et al. 2004) and for migraine headache (Tepper et al. 2001). A phase 11 randomized, double-blind trial of NMMA to treat patients with shock associated with acute myocardial infarction revealed no beneficial or possibly deleterious effects (Alexander et al. 2(X)7). In a different trial, a pro-drug for the NOS2-specific inhibitor L-NIL was administered orally to normal individuals and to those with asthma. The drug reduced exhaled NO and had no effects on blood pressure, pulse, and respiratory function (Hansel et al. 2003). There have been numerous preclinical studies in non-human animals of a variety of nonselective and selective inhibitors. Several NOS 1-specific inhibitors have been studied in animal models of amyotrophic lateral sclerosis, Parkinson s disease, Huntington s disease, Alzheimer s disease, depression, and anxiety (Chabrier et al. 1999). 

O. A. Andreassen, A. Dedeoglu, P. Klivenyi, M. F. Beal, and A. I. Bush. N-acetyl-L-cysteine in )roves survival and preserves motor performance in an animal model of familial amyotrophic lateral sclerosis. Neuroreport 11 (ll) 2491-2493, 2000. 

A. S. Wu, M. Kiaei, N. Aguirre, J. P. Crow, N. Y. Calingasan, S. E. Browne, and M. F. Beal. Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis. TNeurochem. 85 (1) 142-150,2003. 

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