{"id":1092,"date":"2014-06-18T16:43:53","date_gmt":"2014-06-18T23:43:53","guid":{"rendered":"http:\/\/blogs.oregonstate.edu\/linuspaulinginstitute\/?p=1092"},"modified":"2016-05-17T09:52:32","modified_gmt":"2016-05-17T16:52:32","slug":"copper-meets-sod-als-story","status":"publish","type":"post","link":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/2014\/06\/18\/copper-meets-sod-als-story\/","title":{"rendered":"When Copper Meets SOD: An ALS Story"},"content":{"rendered":"

\"DeathThe Linus Pauling Institute has some very exciting research to report on potential therapies for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig\u2019s disease.\u00a0Dr. Joseph Beckman<\/a>\u00a0from the\u00a0LPI\u00a0with\u00a0colleagues in Australia and the United Kingdom have shown that a copper compound, called CuATSM, delayed the onset of symptoms and extended lifespan in mouse models of ALS<\/a>.<\/p>\n

Put simply, this is groundbreaking.<\/i><\/p>\n

<\/strong>Currently, treatment options for ALS are few and not very effective. This disease hits a very specific part of the body: motor neurons \u2013 cells that control every movement. However, motor neurons lie in the protected environment of the spinal cord, making ALS extremely difficult to treat.<\/p>\n

\u00a0<\/span>Another key to the disease is thought to be superoxide dismutase 1 (SOD1). This antioxidant enzyme normally removes harmful oxygen radicals in the body \u2013 at least when it\u2019s working properly.<\/p>\n

When SOD1 is lacking one of its metal atoms (it has one copper and one zinc atom), it \u201cunfolds\u201d and starts causing damage. Instead of helping to protect motor neurons, it becomes toxic.<\/p>\n

Laboratory animals with ALS express mutant forms of SOD1 \u2013 mutations that have been found in humans with the disease. These mutant forms of SOD1 easily lose one of their metal ions, giving animals the same progressive paralysis and wasting seen in ALS patients.<\/p>\n

\n \"Can<\/a>\n
Figure from Roberts et al. J Neurosci. (2014) 34(23):8021-31. doi: 10.1523<\/figcaption>\n <\/figure>\n

One approach to restore SOD1 function is to try forcing the metals back into the enzyme. This is where CuATSM comes into play. Since copper is carefully controlled in the body for a variety of reasons, including potential toxicity, most copper therapies are very difficult to use. CuATSM, however, is capable of delivering copper atoms in hard-to-reach places in the body.<\/p>\n

As described by Dr. Beckman and his colleagues in the\u00a0The Journal of Neuroscience, <\/i><\/a>CuATSM fed to laboratory animals with ALS actually delivered copper to motor neurons of the spinal cord. Although the animals wound up producing more mutant SOD1, the extra copper allowed SOD1 to stay in its properly folded, protective form.<\/p>\n

Best of all, the CuATSM delayed the progression of ALS<\/i> in these animals, and they lived longer than animals that did not receive it.<\/p>\n

So for the first time, a promising therapy may exist for ALS \u2013 and since CuATSM has already been used in humans, it\u2019s not expected to have serious negative effects.<\/p>\n

\"Joseph<\/p>\n

As Dr. Beckman\u00a0said<\/a>, \u201cWe believe that with further improvements, and following necessary human clinical trials for safety and efficacy, this could provide a valuable new therapy for ALS and perhaps Parkinson\u2019s disease.\u201d<\/p>\n

While much more work still needs to be done, these findings are already being heralded as a breakthrough.<\/p>\n

Have any comment to share? Have any more questions about ALS or CuATSM? \u00a0<\/strong><\/p>\n","protected":false},"excerpt":{"rendered":"

The Linus Pauling Institute has some very exciting research to report on potential therapies for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig\u2019s disease.\u00a0Dr. Joseph Beckman\u00a0from the\u00a0LPI\u00a0with\u00a0colleagues in Australia and the United Kingdom have shown that a copper compound, called CuATSM, delayed the onset of symptoms and extended lifespan in mouse models of ALS.… Continue reading →<\/span><\/a><\/p>\n","protected":false},"author":5337,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"jetpack_post_was_ever_published":false,"_jetpack_newsletter_access":"","_jetpack_dont_email_post_to_subs":false,"_jetpack_newsletter_tier_id":0,"_jetpack_memberships_contains_paywalled_content":false,"_jetpack_memberships_contains_paid_content":false,"footnotes":"","jetpack_publicize_message":"","jetpack_publicize_feature_enabled":true,"jetpack_social_post_already_shared":true,"jetpack_social_options":{"image_generator_settings":{"template":"highway","default_image_id":0,"font":"","enabled":false},"version":2}},"categories":[159207],"tags":[],"class_list":["post-1092","post","type-post","status-publish","format-standard","hentry","category-feature-stories"],"jetpack_publicize_connections":[],"jetpack_featured_media_url":"","jetpack_sharing_enabled":true,"jetpack-related-posts":[{"id":1313,"url":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/2016\/02\/03\/als-patients-cuatsm-clinical-trials\/","url_meta":{"origin":1092,"position":0},"title":"Summary for ALS Patients about CuATSM and Clinical Trials","author":"Anne Glausser","date":"February 3, 2016","format":false,"excerpt":"\u00a0 Below is an open letter from LPI researcher Joe Beckman to ALS patients who are interested in hearing more about the next steps of his groundbreaking finding released last week that halted the progression of ALS in mice.\u00a0 \u00a0 Please accept my apologies for writing a general note.\u00a0 The\u2026","rel":"","context":"In "Ask the Expert"","block_context":{"text":"Ask the Expert","link":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/category\/ask-the-expert\/"},"img":{"alt_text":"Joe Beckman, PhD - ALS reseacher","src":"https:\/\/i0.wp.com\/blogs.oregonstate.edu\/linuspaulinginstitute\/files\/2016\/02\/beckmanmedium.jpg?resize=350%2C200","width":350,"height":200},"classes":[]},{"id":1925,"url":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/2017\/03\/14\/update-als-clinical-trials\/","url_meta":{"origin":1092,"position":1},"title":"An Update on Copper-ATSM and Clinical Trials for ALS, Updated March 2017","author":"Alexander Michels, PhD","date":"March 14, 2017","format":false,"excerpt":"Friends and followers of the Linus Pauling Institute, My name is Joseph Beckman, a principal investigator at the Linus Pauling Institute that works on ALS (aka Lou Gehrig\u2019s disease). Without repeating myself too much, I would like to point everyone to a\u00a0summary of our advances in ALS research that came\u2026","rel":"","context":"In "Ask the Expert"","block_context":{"text":"Ask the Expert","link":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/category\/ask-the-expert\/"},"img":{"alt_text":"Copper ATSM molecular structure","src":"https:\/\/i0.wp.com\/blogs.oregonstate.edu\/linuspaulinginstitute\/files\/2017\/03\/Artboard-1-300x175.png?resize=350%2C200","width":350,"height":200},"classes":[]},{"id":1358,"url":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/2016\/02\/26\/diet-for-a-healthy-heart\/","url_meta":{"origin":1092,"position":2},"title":"Diet for a Healthy Heart","author":"Giana Angelo","date":"February 26, 2016","format":false,"excerpt":"You\u2019ve probably heard it before, but it\u2019s worth repeating: Heart disease is the leading cause of death for both men and women Diet and lifestyle choices can have a dramatic effect on the development of heart disease and other conditions affecting the cardiovascular system It\u2019s never too late to start\u2026","rel":"","context":"In "Ask the Expert"","block_context":{"text":"Ask the Expert","link":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/category\/ask-the-expert\/"},"img":{"alt_text":"A Healthy Heart of Fruits and Vegetables","src":"https:\/\/i0.wp.com\/blogs.oregonstate.edu\/linuspaulinginstitute\/files\/2016\/02\/DOH-Heart-300x266.png?resize=350%2C200","width":350,"height":200},"classes":[]},{"id":970,"url":"https:\/\/dev.blogs.oregonstate.edu\/linuspaulinginstitute\/2015\/09\/04\/fish-oil-good-health\/","url_meta":{"origin":1092,"position":3},"title":"Fish oil: Good for my health… or not?","author":"Giana Angelo","date":"September 4, 2015","format":false,"excerpt":"Stories from various news outlets (and even from different researchers at Oregon State University) have said: Fish oil\/omega-3s are great! Excess omega-3s are really bad! Fish oil\/omega-3s are great\u00a0(again)!! Never mind,\u00a0fish oil doesn\u2019t work... This is confusing and frustrating for all of us. 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