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Amyotrophic Lateral Sclerosis (ALS)
Also called: ALS, Lou Gehrig's disease
Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons transmit messages from your brain and spinal cord to your voluntary muscles - the ones you can control, like in your arms and legs. At first, this causes mild muscle problems. Some people notice
• Trouble walking or running
• Trouble writing
• Speech problems
• Eventually, you lose your strength and cannot move. When muscles in your chest fail, you cannot breathe. A ventilator can help, but most people with ALS die from respiratory failure. The disease usually strikes between age 40 and 60. More men than women get it. No one knows what causes ALS. It can run in families, but usually it strikes at random. There is no medical cure. Medicines can relieve symptoms and, sometimes, prolong survival.
Collagen cross-linking of skin in patients with amyotrophic lateral sclerosis.
Ann Neurol. 1992 Mar;31(3):305-10.
Ono S, Yamauchi M.
Dental Research Center, University of North Carolina 27599-7455.
Collagen cross-links of skin tissue (left upper arm) from 11 patients with amyotrophic lateral sclerosis (ALS) and 9 age-matched control subjects were quantified. It was found that patients with ALS had a significant reduction in the content of an age-related, stable cross-link, histidinohydroxylysinonorleucine, that was negatively correlated with the duration of illness. The contents of sodium borohydride-reducible labile cross-links, dehydro-hydroxylysinonorleucine and dehydro-histidinohydroxymerodesmosine, were significantly increased and were positively associated with the duration of illness (r = 0.703, p less than 0.05 and r = 0.684, p less than 0.05, respectively). The results clearly indicate that during the course of ALS, the cross-linking pathway of skin collagen runs counter to its normal aging, resulting in a "rejuvenation" phenomenon of skin collagen. Thus, cross-linking of skin collagen is affected in ALS.
Studies:
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