Zone in with Zon–Focus on ALS (Lou Gehrig’s Disease)

Dr. Gerald Zon’s latest “Zone in with Zon” blog post, September 3, 2014, describes the origin and history of the recent phenomenally successful fund-raising effort known as the ALS Ice Bucket Challenge, as well as the current status of research on this terrible disease. For those of you who don’t know, the Ice Bucket Chalenge is an effort to raise funds for research on the disease amyotrophic lateral sclerosis (ALS), which killed the famous baseball player Lou Gehrig and is also termed “Lou Gehrig’s disease.” The ALS Ice Bucket Challenge involves dumping a bucket of ice water on someone’s head to promote awareness of ALS and to encourage donations—typically $100—to research on the disease. The challenge dares nominated participants to be recorded having a bucket of ice water poured on their heads, and challenging others to do the same. A common stipulation is that nominated people have 24 hours to comply or forfeit by way of a charitable financial donation. Although there is certainly an aspect of fun to this effort, Dr. Zon points out the grim reality that there is presently no cure for this deadly disease that affects 350,000 people around the globe and kills 100,000 annually. He further notes that the disease can impact anyone anywhere, regardless of age, ethnicity, or socioeconomic background. Although usually rapidly fatal, ALS does not always kill quickly. Famous British physicist Stephen Hawking has lived with ALS for many years and is now 72 years of age. Dr. Zon briefly discusses what is known about the genetics of ALS, while giving the caveat that the genetics appears very complex. He notes that a defect in the gene for superoxide dismutase (SOD) on chromosome 21 is associated with approximately 20% of familial ALS and approximately 2% of ALS cases overall, that is including sporadic or non-familial cases. Dr.
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