Neuronal Diseases

Neurological diseases belong to a class of acute or chronic diseases which are characterized by neuronal injury or neuronal loss. They include spinal muscular atrophy, amyotrophic lateral sclerosis and brain cancer. Gene therapy has opened a new way to treat these diseases. A starshaped polymer consisting of a p-CD core and poly(amidoamine) (PAMAM) was synthesized as a vector to transfect the human neuroblastoma SH-SY5Y cells and demonstrated lower toxicity compared to those of the PAMAM/ pDNA complex.  

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Mulder, D.W. (1986). Motor neuron disease in adults. In Myology Volume II (Engel, A.G., Banker. 

W0 9 413 288 (Rhone-PoulencRorer appl. 10.12.1993 F-prior. 16.12.1992). use to treat motor-neuron diseases ... 

Motor neuron disease AIDS Subacute Motor dysfunction, muscle wasting with upper motor neuron signs bulbar dysfunction Possible immune mediated... 

Galassi G, Gentilini M et al (1998) Motor neuron disease and HIV-1 infection in a 30-year-old HIV-positive heroin abuser a causal relationship Clin Neuropathol 17(3) 131-135 Gherardi R, Lebargy F et al (1989) Necrotizing vasculitis and HIV replication in peripheral nerves. N Engl J Med 321(10) 685-686... 

Schuffler MD, Bird TD, Sumi M, Cook A A familial neuronal disease presenting as intestinal pseudoobstruction. Gastroenterology 1978 75 889-898. 

Human creatine kinase -MM MAK33 IgGl Cardiac disease, mitochondrial disorders, inflammatory myopathies, myasthenia, polymyositis, McArdle s disease, NMJ disorders, muscular dystrophy, ALS, hypo and hyperthyroid disorders, central core disease, acid maltase deficiency, myoglobinuria, rhabdomyolysis, motor neuron diseases, A. thaliana A. thaliana 2S2 seed storage protein SP + 0.02-0.4% TSP of fresh leaf extract (10-12% TSP of intercellular fluid) 52... 

DISORDERS OF MUSCLE EXCITABILITY 713 MOTOR NEURON DISEASES 731... 

AMYOTROPHIC LATERAL SCLEROSIS IS THE MOST COMMON ADULT ONSET MOTOR NEURON DISEASE 731... 

Motor neuron disease is characterized clinically by weakness, muscle atrophy and spasticity 732... 

NON-TRANSGENIC, INDUCED MODELS OF MOTOR NEURON DISEASE 734... 

Transgenic mice expressing wild type or mutant neurofilament genes develop motor neuron disease and neurofibrillary pathology 736... 

The motor neuron diseases (MND), including amyotrophic lateral sclerosis (ALS), are chronic, progressive illnesses characterized by severely disabling clinical features... 

Motor neuron disease is characterized clinically by weakness, muscle atrophy and spasticity. This illness, often termed Lou Gehrig s disease in the United States, is the most common adult-onset form of MND with a prevalence of approximately 2-3 per 100,000 people [1-3,10, 25, 28]. Each year in the United States, in excess of 5,000 people are diagnosed with ALS. In parts of the United Kingdom, 1 in =500 deaths are attributed to some form of MND. The principal clinical signs of ALS include progressive limb weakness, which may be symmetrical or asymmetrical atrophy of appendicular, bulbar and respiratory muscles and spasticity [1,2,26,28]. The paralysis/muscle atrophy and spasticity are the result of degeneration of motor neurons in the spinal cord/brain stem and motor cortex respectively. The onset of this illness is typically in the fifth or sixth decade of life affected individuals usually... 

Wong, P. C., Pardo, C. A., Borchelt, D. R. et al. An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14 1105-1116,1995. 

Subramaniam, J. R., Lyons, W. E., Liu, J. et al. Mutant SOD1 causes motor neuron disease independent of copper chaperone- mediated copper loading. Nat. Neurosci. 5 301-307, 2002. 

Wong, P. C., Rothstein, J. D. and Price, D. L. The genetic and molecular mechanisms of motor neuron disease. Curr. Opin. Neurobiol. 8 791-799,1998. 

Carpenter, S. Proximal axonal enlargement in motor neuron disease. Neurology 18 841-851,1968. 

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. 

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