Amyotrophic lateral sclerosis motor neurons

Orrell RW, Lane RJM, Ross M (2005) Andoxidant treatment for amyotrophic lateral sclerosis/motor neuron disease. The Cochrane Database for Systemadc Reviews, Volume 4, The Cochrane Col-labor adon. 

Lou Gehrig s disease (amyotrophic lateral sclerosis ALS) displays motor neuron deposits of hyperphosphorylated neurofilament subunits in the sporadic disease. Familial ALS, some 20% of all cases of ALS, involves dominant superoxide dismutase SOD1 mutants that can form (3-barrel aggregates [49-51]. 

The lack of zinc can also be a problem in biological systems and is responsible for disease states. For example, nitric oxide-dependent apoptosis can be induced in motor neurons by zinc-deficient SOD, and in some cases of amyotrophic lateral sclerosis, zinc-deficient SOD may participate in this type of oxidative mechanism involving nitric oxide.969 One form of hereditary human hair loss or alopecia was mapped to a specific gene and a mutation found in affected individuals. The gene encodes a single zinc finger transcription factor protein with restricted expression in the brain and skin.970 Zinc has been implicated in Alzheimer s via beta amyloid formation, and a role has been attributed for the cerebral zinc metabolism in the neuropathogenesis of Alzheimer s disease.971... 

IGF I has recently been the focus of considerable interest due to its actions on motor neurons. It can prevent normal motor neuron cell death during development, reduce the loss of these cells following nerve injury and enhance axonal regeneration. In the adult, injection of IGF I results in sprouting of motor neuron terminals and increases the size of the neuromuscular junction. These and other studies suggest potential therapeutic applications of IGF I in several neurological diseases including amyotrophic lateral sclerosis and peripheral neuropathies. 

Diseases selectively targeting spinal cord and brainstem motor neurons (e.g. amyotrophic lateral sclerosis and the familial spinal muscular atrophies) or the presynaptic component of neuromuscular junctions (e.g. Lambert-Eaton syndrome, botulism and Ixodes tick paralysis) cause weakness without sensory impairment. Disorders involving the enteric nervous system (e.g. Chagas disease and Hirschsprung s disease) impair bowel motility. 

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

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

Amyotrophic lateral sclerosis 2 is linked to mutant alsin. In several families with autosomal recessive juvenile ALS, mutations have been identified in ALS2 (chromosome 2), encoding alsin [65, 66], This illness, which was originally described in a Tunisian kindred, is characterized by spasticity (involvement of upper motor neurons) and weakness/amyotrophy (involvement of lower... 

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. 

Williamson, T. L., Bruijn, L. I., Zhu, Q. et al. Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant. Proc. Natl Acad. Sci. U.S.A. 95 9631-9636,1998. 

Amyotrophic lateral sclerosis (ALS) is a degenerative disorder of motor neurones. In 15-25% of cases, the genetic cause of the disease is a mutation of the enzyme Cu+/Zn2+... 

One group of people who may be at greater risk are those who are exposed to cyanide but are unable to smell the chemical (Kirk and Stenhouse 1953 Snodgrass 1996). Patients with motor neuron disease (amyotrophic lateral sclerosis) possess a disorder in cyanide detoxification that may result in their higher susceptibility to cyanide (Kato et al. 1985). 

Frontotemporal dementia involves an early and primary degenerative process of frontal and/or temporal cortex. Several disorders fall under this rubric, such as Pick s disease and the dementia associated with amyotrophic lateral sclerosis (ALS). ALS is a degenerative disease of upper motor neurons that is sometimes accompanied by a frontal lobe dementia (Vercelletto et al. 1999 Abe et al. 1997). ALS has been associated with mutations in the free radical scavenging enzyme superoxide dismutase 1 (Price et al. 1997). Pick s disease is associated histologically with a loss of neurons and cytoplasmic Pick bodies in surviving neurons. 

Jiang YM, Yamamoto M, Kobayashi Y, Yoshihara T, Liang Y, Terao S, Takeuchi H, Ishigaki S, Katsuno M, Adachi H, Niwa J, Tanaka F, Doyu M, Yoshida M, Hashizume Y, Sobue G (2005) Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Ann Neurol 57(2) 236-251 Johnstone RW (2002) Histone-deacetylase inhibitors novel dmgs for the treatment of cancer. Nat Rev Dmg Discov l(4) 287-299... 

Amyotrophic lateral sclerosis (ALS) is a disease of the neurons that control muscle movement (motor neurons). Degeneration of neurons causes muscle atrophy eventually impairing the movement of people afflicted with the disease. 

Motor neurone disease is often called amyotrophic lateral sclerosis in the USA, where it is also known as Lou Gehrig s disease after the famous New York Yankees baseball player who developed the disease in 1939. 

Peripheral neuropathies Amyotrophic lateral sclerosis Alzheimer s disease Parkinson s disease Huntington s disease Sensory/motor neurons Spinal motor neurons Forebrain cholinergic neurons Nigral dopamine neurons Striatal GABA neurons NGF, IGF-1, NT-3, CNTF BDNF, IGF-1, CNTF, GDNF, NT-4/5 NGF, BDNF, NT-4/5 GDNF, BDNF, NT-3, NT-4/5 BDNF, NT-4/5... 

Amyotrophic lateral sclerosis (ALS) is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons in the central nervous system. No cure has yet been found for ALS. The U.S. Food and Drug Administration (FDA) has approved riluzole as the first drug treatment for the disease. It delays the onset of ventilator-dependence or tracheostomy in selected patients. A Cochrane review states a 9% gain in the probability of surviving one year (see Miller et ah, 2007). 

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