Friday, February 21, 2014

Nervous System Support Cells are Toxic to Motor Neurons in Sporadic ALS Model

Washington, D.C. (February 21, 2014)—In work supported by The ALS Association, scientists have discovered new insights into the causes of motor neuron death in ALS. The findings are likely to lead to testing of new therapeutic strategies to slow the progression of the disease. The study was published in the journal Neuron.

ALS (amyotrophic lateral sclerosis), otherwise known as Lou Gehrig’s Disease, is a progressive neurodegenerative disease that affects neurons (nerve cells) in the brain and the spinal cord. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. There is no cure and no life-prolonging treatments for the disease. 
Previous work has shown that an unknown toxic property of supporting cells, called astrocytes, contributes to the death of motor neurons in ALS. Much of that work has been done in a disease model with artificially elevated levels of the ALS-causing protein, superoxide dismutase (SOD). To test whether a similar mechanism was at work without that protein elevation, researchers grew astrocytes from ALS patients with the sporadic (non-familial) form of the disease and exposed them to human motor neurons from healthy embryonic stem cells. This is one of the first times that such a “fully humanized” model of sporadic ALS combining astrocytes and motor neurons has been produced.
The researchers found that motor neurons succumbed to the toxic effects of the astrocytes, through a specific process of cell death called necroptosis. Because this process is at least partially understood on the molecular level, it should be possible to design treatments to interrupt it, which may be therapeutic.
The research was performed by Diane Re, Ph.D., and Virginia Le Verche, Ph.D., under the leadership of Serge Przedborski, M.D., Ph.D., all of Columbia University in New York and was funded by The ALS Association, Greater New York Chapter.
“These exciting results shed new light on the interaction between motor neurons and astrocytes,” said Lucie Bruijn, Ph.D., Chief Scientist for The Association. “The finding that astrocytes from sporadic ALS patients exert toxicity will strengthen the focus on understanding this interaction more closely and point out possible new targets for therapy.”

In Sporadic ALS Model, Nervous System Support Cells Are Toxic to Motor Neurons

February 21, 2014

Scientists at Columbia University in New York have discovered new insights into the causes of motor neuron death in ALS. These findings are likely to lead to testing of new therapeutic strategies to slow the disease’s progression.
Past work has shown that an unknown toxic property of supporting cells known as astrocytes contributes to the death of motor neurons in ALS. Much of that work has been done in a disease model with artificially elevated levels of the ALS-causing protein, superoxide dismutase (SOD). To test whether a similar mechanism was at work without that protein elevation, the researchers at Columbia grew astrocytes from individuals with ALS who have the sporadic (non-familial) form of the disease and exposed them to human motor neurons from healthy embryonic stem cells. This is one of the first times that scientists have produced a “fully humanized” model of sporadic ALS that combines astrocytes and motor neurons.
The researchers discovered that motor neurons succumbed to the toxic effects of the astrocytes, through a specific process of cell death called necroptosis. Because this process is at least partially understood on the molecular level, it should be possible to design treatments to interrupt it, which may be therapeutic.
The research was performed by Diane Re, Ph.D., and Virginia Le Verche, Ph.D., under the leadership of Serge Przedborski, M.D., Ph.D., and was funded by The ALS Association, Greater New York Chapter. The study appears in the journal Neuron.
“These exciting results shed new light on the interaction between motor neurons and astrocytes,” said Lucie Bruijn, Ph.D., Chief Scientist for The Association. “The finding that astrocytes from sporadic ALS patients exert toxicity will strengthen the focus on understanding this interaction more closely and point out possible new targets for therapy.”

The Califor(five,six)nia Proposal


The Califor(five,six)nia Proposal

Could new states be on the way?
Could new states be on the way?
It seems as if wildfires and earthquakes will face unexpected competition in the process of disfiguring California.  Tim Draper, venture capitalist and co-founder of Draper Fisher Jurveston, recently launched a campaign to divide California into six separate states, irrevocably altering the “Fifty Nifty United States”.  The California native has commenced the initial steps to have his proposal included on the November 2014 state ballot.
Draper is the alleged culprit behind leaking a draft of the proposal in December, as identified to be authored by a group of venture capitalists.  The contents of the proposal begin by stating the issues leading to their call for action.  First, the writers state California’s population of 38 million is six times the national state population average.  While this statistic is difficult to interpret due to population dispersion, it’s true that California alone makes up 12 percent of the U.S. population.  Additionally, the proposal claims the state’s geographic enormity and its spectrum of diversity from North to South are matters of great consideration.  The writers also document that in 1859, a vast majority of the voters were in favor of splitting California into two states.  In reaction to disproportionate taxes favoring the North, Ranchero Don Andres Pico proposed the Pico Act to split the state into two at the 36th parallel.  His plan won state Legislative and Executive approval in less than one month, leaving only a need for the national government’s assent.  However, the significance of the Pico Act quickly diminished due to the eruption of the Civil War.  Thus, the proposal died federally as quickly as it thrived regionally.
Hoping for success where history once failed, Draper’s motion calls for a six-way divide of the state.  From North to South, the states would be as follows: Jefferson, North California, Central California, Silicon Valley (Draper’s native land), West California, and South California.  To guarantee the success of implementation, Draper proposes the establishment of a 24-member Board of Commissioners who would be responsible for dispersing California’s funds and property among the newly created states within two years.  And finally, in striving for a favorable outcome, Draper deems himself the “appointed ‘agent of the State of California’” in the process of defending the initiative against legal backlashes.
Because the proposal is so radical, it faces strong resistance by contenders even in its earliest stages of development.  First and foremost, many argue this is a clear violation of Article 4, Section 3 of the U.S. Constitution, which states, “No new State shall be formed or erected within the Jurisdiction of any other State”.  The counterargument, though, is to point to the secessions of West Virginia and Nevada from Confederate states during the Civil War.
Allowing that this is legally plausible, then, the argument becomes whether it is possible for this severance to occur neatly and fairly. For example, certain legalities exist uniquely under California law, which need to be addressed concerning the transition period.  Matters of concern include water rights, distribution of state universities, and the new interstate commerce that would arise out of what is currently state commerce.  From the perspective regarding social policy, the question arises: would each individual state be allowed to choose their stance on the legalizations of same sex marriage and marijuana, or would they immediately assume California’s single-state stances?  The eastern border of the state is largely conservative, so the latter could cause a serious setback in actualizing the proposal.  In fact, conservatism is a major cause of concern for Californian Democrats.  Considering two senators would represent each new territory, this could expand Republican representation beyond its current bounds and, in effect, cause problems gaining Democratic congressional support for the severance.  But according to Draper, this hindering political divide is exactly what his proposal warrants against.  In an email he sent to TechCrunch in December, Draper claimed the state is “ungovernable” given its current borders.  His solution is to gain what he considers fair representation within in the Senate for all Californians.
Despite Draper’s good intentions, a December poll revealed only 25 percent of California residents support his proposal while 59 percent are in opposition.  Expectedly, the majority of his support derives from less populous areas of California who actively fight against large cities like Los Angeles and San Francisco gaining political domination despite little geographic representation.  While the proposal addresses this issue by improving legislative representation, other size concerns aren’t being addressed.  Because the proposal suggests state funds should be dispersed based on population, legislative analysts have predicted the formation of two wealthy states and four poor.  The result, then, is the current problem: underrepresentation as a Californian entity for rural areas.
Do the consequences of the proposal outweigh the benefits?  Draper certainly doesn’t think so and is devoting his deep pockets to prove it.  His six-state petition requires one million signatures in order to even be presented on the upcoming autumn ballot, so promotion of the idea will certainly require his financial backing.  Fortunately, his million dollar successes in starting up Hotmail, Skype, and his own venture capitalist firm provide him with the legitimacy required to tackle such a tremendous task.  So far, public advocacy is limited to periodic news interviews and a website currently in beta, but Draper has plans to increase awareness in hopes of convincing his fellow Californians and, in the long run, his fellow Americans that it is best to be united citizens of the 55 states of America.

Wednesday, February 12, 2014

I Fought For You

http://www.youtube.com/v/AgYLr_LfhLo?version=3&hl=en_US&rel=0