Common Cause of All Forms of Amyotrophic Lateral Sclerosis (ALS) Discovered

[n2doc]

ScienceDaily (Aug. 21, 2011) — The underlying disease process of amyotrophic lateral sclerosis (ALS and Lou Gehrig's disease), a fatal neurodegenerative disease that paralyzes its victims, has long eluded scientists and prevented development of effective therapies. Scientists weren't even sure all its forms actually converged into a common disease process.

But a new Northwestern Medicine study for the first time has identified a common cause of all forms of ALS.

The basis of the disorder is a broken down protein recycling system in the neurons of the spinal cord and the brain. Optimal functioning of the neurons relies on efficient recycling of the protein building blocks in the cells. In ALS, that recycling system is broken. The cell can't repair or maintain itself and becomes severely damaged.

The discovery by Northwestern University Feinberg School of Medicine researchers, published in the journal Nature, provides a common target for drug therapy and shows that all types of ALS are, indeed, tributaries, pouring into a common river of cellular incompetence.

more

http://www.sciencedaily.com/releases/2011/08/110821141115.htm

[livetohike]

1. Thanks for posting this. I lost an Aunt to ALS this year...n/t

[DesertRat]

2. This is encouraging news.

Hopefully this new discovery will lead scientists closer to a cure.

[Happyhippychick]

3. My dad died from this horrifying disease in 2005. Thanks for posting!

[Warpy]

4. I lost a cousin from this disease a few years ago.

It really is a horrible way to go, the mind remaining intact while the body goes completely out of control. She had the central variety, so it was six months from diagnosis to a miserable death.

[laconicsax]

5. On the MSN homepage the other day...

There was a link that read, "What disease is named for Lou Gehrig?"

[Duncan Grant]

6. K&R (nt)

:kick:

[Celebration]

7. ALS and statins

http://www.spacedoc.com/ALS_statins.html

Another report was recently sent to me of amyotrophic lateral sclerosis (ALS) associated with the use of statin drugs. There is not the slightest doubt in my mind that the numbers of reports I am seeing now are far more than usually expected in a group the size of my reporting population. One naturally wonders about this curious relationship with statin drugs and what the possible mechanism of action might be.

Recently a neuroscientist, V. Meske, reported in the European Journal of Neuroscience a very relevant study about the ability of statin drugs to cause neuronal degeneration. Statin drugs are designed to inhibit cholesterol synthesis by their effect on the mevalonate pathway.

It seems that a consequence of the inhibitory effect of statin drugs on the mevalonate pathway is the induction of abnormal tau protein phosphorylation.Tau protein phosphorylation goes on to form neurofibrillatory tangles, long known to be the prime suspect in causing the slowly progressive neuronal degeneration of Alzheimer's disease.

Sometimes this process is accompanied by beta amyloid deposition but more commonly not. Research scientists are now finding that this mechanism appears to be true for ALS and many other forms of neurodegenerative diseases as well. They have even coined a new word for this, the taupathies.

[HysteryDiagnosis]

8. The quacks at the American Journal of Epidemiology say.....

http://www.ncbi.nlm.nih.gov/pubmed/21335424

Am J Epidemiol. 2011 Mar 15;173(6):595-602. Epub 2011 Feb 18.
Vitamin E intake and risk of amyotrophic lateral sclerosis: a pooled analysis of data from 5 prospective cohort studies.
Wang H, O'Reilly ÉJ, Weisskopf MG, Logroscino G, McCullough ML, Schatzkin A, Kolonel LN, Ascherio A.
Source

Department of Nutrition, Harvard School of Public Health, Boston, Massachusetts 02115, USA.
Abstract

The authors investigated whether vitamin E intake was associated with amyotrophic lateral sclerosis (ALS) in the Nurses' Health Study (1976-2004), the Health Professionals Follow-up Study (1986-2004), the Cancer Prevention Study II Nutrition Cohort (1992-2004), the Multiethnic Cohort Study (1993-2005), and the National Institutes of Health-AARP Diet and Health Study (1995-2005). ALS deaths were identified through the National Death Index. In the Nurses' Health Study and the Health Professionals Follow-up Study, confirmed nonfatal ALS cases were also included. Cohort-specific results were estimated using Cox proportional hazards models and pooled using random-effects models.

Among 1,055,546 participants, 805 developed ALS. Overall, using vitamin E supplements was not associated with ALS. However, within cohorts with information on duration of vitamin E supplement use (231 cases), ALS rates declined with increasing years of use (P-trend=0.01). Compared with nonusers, the multivariable-adjusted relative risk was 1.05 (95% confidence interval (CI): 0.60, 1.84) among users for ≤1 year (12 cases), 0.77 (95% CI: 0.33, 1.77) among users for 2-4 years (7 cases), and 0.64 (95% CI: 0.39, 1.04) among users for ≥5 years (18 cases).

For dietary vitamin E intake, the multivariable-adjusted relative risk comparing the highest quartile with the lowest was 0.79 (95% CI: 0.61, 1.03); an inverse dose-response was evident in women (P-trend=0.002) but not in men (P-trend=0.71). In this large, pooled prospective study, long-term vitamin E supplement use was associated with lower ALS rates. A possible protective effect of vitamin E deserves further consideration.