Amylotrophic lateral sclerosis

Neurological diseases Huntington s disease (A), amylotrophic lateral sclerosis (A,I), Parkinson s disease (A), traumatic brain injury (A,I), glaucoma (A)... 

In addition to the cytosolic SOD there is a longer 222-residue extracellular form that binds to the proteoglycans found on cell surfaces.522,527 Manganese SODs are found in mitochondria and in bacteria and iron SODs in plants and bacteria. They all appear to be important in protecting cells from superoxide radicals.522/532/533 This importance was dramatically emphasized when it was found that a defective SOD is present in persons (about 1 in 100,000) with a hereditary form of amylotrophic lateral sclerosis (ALS), which is also called Lou Gehrig s disease after the baseball hero who was stricken with this terrible disease of motor neurons in 1939 at the age of 36.534-536... 

Spencer, P. S., P. B. Nunn, J. Hugon, A. C. Ludolph, S. M. Ross, D. N. Roy, and R. C. Robertson, Guam amylotrophic lateral sclerosis—Parkinsonism—Dementia linked to a plant excitant neurotoxin. Science, 237, 517-522 (1987). 

The neurological disease amylotrophic lateral sclerosis (ALS), also called Lou Gehrig s disease, results in paralysis as nerve cells are progressively destroyed. It was found that spinal cord cells accumulate brain lipids and in particular ceramide (Cutler et al, 2002). This accumulation can be abrogated by treatment with ISP-1, an inhibitor of serine palmitoyltransferase, and this inhibitor attenuated cellular damage in an in vitro model of ALS (see Table 2). 

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