Tuesday, August 19, 2014

ALS findings - what do we know?

Amid the flood of ALS bucket challenges, I thought I'd take a moment to google for what's known. Here's what I found:

What is ALS?

A-myo-trophic comes from the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment–"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement.

- http://www.alsa.org/about-als/what-is-als.html

What causes ALS?

August 21, 2011 - Ubiquilin2 in spinal and brain system cells is supposed to repair or dispose of other proteins as they become damaged. The researchers discovered a breakdown of this function in ALS patients.

When Ubiquilin2 is unable to remove or repair damaged proteins, the damaged proteins begin to pile up in the cells, eventually blocking normal transmission of brain signals in the spinal cord and brain, leading to paralysis.

- http://articles.chicagotribune.com/2011-08-21/news/chi-northwestern-research-unveils-clues-to-cause-of-als-20110821_1_als-patients-key-protein-northwestern-study

April 4, 2014 - "Like the studs, joists and rafters of a house, the neurofilament is the backbone of the cell, but it's constantly changing. These proteins need to be shipped from the cell body, where they are produced, to the most distant part, and then be shipped back for recycling," he said. "If the proteins cannot form correctly and be transported easily, they form tangles that cause a cascade of problems."

Zhang's research group found "the disease ALS is caused by misregulation of one step in the production of the neurofilament," he said.

- http://www.latinpost.com/articles/10061/20140404/cause-of-als-lou-gehrigs-disease-found.htm

What is causing the protein transport process to fail?

January 05, 2012 - ...a serendipitous trail of clues led him to discover that a tiny toxic molecule, beta-methylamino-L-alanine (BMAA), believed to be from cycads on Guam, was in fact produced by cyanobacteria, and not just on Guam, but around the world. More astonishing, he and Banack discovered that BMAA had accumulated in the brains of humans who’d died from ALS, Alzheimer’s, or Parkinson’s — but not in the brains of people who’d died from other causes.

- http://www.psmag.com/navigation/health-and-behavior/was-lou-gehrigs-als-caused-by-tap-water-38804/

March 31, 2013 - ...folks with ALS have more heavy metal exposures like lead.

In addition, people with ALS have more hydrocarbon exposures like jet fuel, as well as paint strippers, cutting, cooling and lubricating oils, stock-piled anti-freeze, de-icers or coolants like propylene glycol and even dry cleaning agents, auto exhaust, and chlorinated hydro­carbons like pesticides.

Fang F, et al, Workplace exposures and the risk of amyotrophic lateral sclerosis, Environ Health Persp, 117:1387-92, 2009

Kamel f, et al, Lead exposure as a risk factor for amyotrophic lateral sclerosis, Neurodegen Dis, 2:195-201, 2005

Homer RD, et al., Amyotrophic lateral sclerosis among 1991 Gulf War veterans: evidence for a time-limited outbreak, Neuroepidemiol 31:28-32, 2008

Ritchie GD, et al, Effects of repeated exposure to JP-8 jet fuel va­por on learning of simple and difficult operant tasks by rats, J Toxi­col Environ Health A 64:385-417, 2008

- http://www.sandozchiropractic.com/studies-show-amyotrophic-lateral-sclerosis-als-may-be-caused-by-environmental-toxins/