Stellar

scinerds: Research suggests new approach for spinal muscular atrophy ALS drug effects highlights potassium channel role There is no specific drug to treat spinal muscular atrophy (SMA), a family of motor neuron diseases that in its most severe form is the leading genetic cause of infant death in the United States and affects one in 6,000 people overall. But a new multispecies study involving a drug that treats amyotrophic lateral sclerosis (ALS) has pinpointed a mechanism of SMA that drug developers might be able to exploit for a new therapy. The research, published in the Journal of Neuroscience, reports that the drug Riluzole advanced neural cell development in a mammalian model of SMA and restored neuromuscular function and mobility in a Caenorhabditis elegans worm model of the disease. Riluzole has already been tested as a therapy in a very small study of severely affected SMA patients. It failed to help. Nevertheless, what makes the new research encouraging, said Anne Hart, professor of neuroscience at Brown and senior author on the paper, is that the study traces the beneficial action of Riluzole to specific “SK2” potassium channels in worm neurons. Humans have these channels too, and if they can be more precisely targeted by a new drug, she said, that could make a more meaningful difference, at least for some patients. “We’re not suggesting based on this that SMA patients should ask their doctors for Riluzole,” said Hart, who is affiliated with the Brown Institute for Brain Science, “but we are suggesting that this pathway would be useful for therapeutic development.” How Riluzole works Because SMA has a lot in common with ALS, Hart thought Riluzole might still be worth studying in the context of SMA. To do so, she partnered with fellow researchers at Boston Children’s Hospital. They worked in mouse neuronal cells while her team at Brown worked in the worms. For each system, the researchers created SMA models in different ways by disabling the gene that produces the survival motor neuron (SMN) protein. Depletion of that protein causes SMA in people too. In the mammalian neuronal cells, the Children’s Hospital researchers found that Riluzole promoted the growth of axons that was lacking in the SMN-depleted cells. However, Riluzole did this not by increasing SMN levels. Instead, the researchers found evidence that drug treatment matured the neurons more quickly in normal cells. Most attempts to treat SMA have relied on trying to maintain or restore higher levels of SMN, Hart noted. But Riluzole, or a future drug, may instead be able to work by accelerating cell maturity. In the worms meanwhile, the Brown researchers found that Riluzole restored two important neuromuscular behaviors of SMA worms: the pumping action that allows the worms to move food through their digestive tracts and the body bending that they perform when swimming. To learn how Riluzole had this effect, they performed further experiments testing various potassium channels, including SK2, that Riluzole is known to act upon. Losing these channels didn’t cause more problems in animals with less SMN protein, but losing the SK2 potassium channels in particular made neuromuscular function worse. Without the SK2 channels, the drug Riluzole didn’t improve function. “This told us that Riluzole improves motorneuron function by acting through SK2 channels, which we did not know before,” said lead author Maria Dimitriadi, a postdoctoral researcher in Hart’s group. “This is important bec

scinerds:

Research suggests new approach for spinal muscular atrophy

ALS drug effects highlights potassium channel role

There is no specific drug to treat spinal muscular atrophy (SMA), a family of motor neuron diseases that in its most severe form is the leading genetic cause of infant death in the United States and affects one in 6,000 people overall. But a new multispecies study involving a drug that treats amyotrophic lateral sclerosis (ALS) has pinpointed a mechanism of SMA that drug developers might be able to exploit for a new therapy.

The research, published in the Journal of Neuroscience, reports that the drug Riluzole advanced neural cell development in a mammalian model of SMA and restored neuromuscular function and mobility in a Caenorhabditis elegans worm model of the disease.

Riluzole has already been tested as a therapy in a very small study of severely affected SMA patients. It failed to help. Nevertheless, what makes the new research encouraging, said Anne Hart, professor of neuroscience at Brown and senior author on the paper, is that the study traces the beneficial action of Riluzole to specific “SK2” potassium channels in worm neurons. Humans have these channels too, and if they can be more precisely targeted by a new drug, she said, that could make a more meaningful difference, at least for some patients.

“We’re not suggesting based on this that SMA patients should ask their doctors for Riluzole,” said Hart, who is affiliated with the Brown Institute for Brain Science, “but we are suggesting that this pathway would be useful for therapeutic development.”

How Riluzole works

Because SMA has a lot in common with ALS, Hart thought Riluzole might still be worth studying in the context of SMA. To do so, she partnered with fellow researchers at Boston Children’s Hospital. They worked in mouse neuronal cells while her team at Brown worked in the worms.

For each system, the researchers created SMA models in different ways by disabling the gene that produces the survival motor neuron (SMN) protein. Depletion of that protein causes SMA in people too.

In the mammalian neuronal cells, the Children’s Hospital researchers found that Riluzole promoted the growth of axons that was lacking in the SMN-depleted cells. However, Riluzole did this not by increasing SMN levels. Instead, the researchers found evidence that drug treatment matured the neurons more quickly in normal cells.

Most attempts to treat SMA have relied on trying to maintain or restore higher levels of SMN, Hart noted. But Riluzole, or a future drug, may instead be able to work by accelerating cell maturity.

In the worms meanwhile, the Brown researchers found that Riluzole restored two important neuromuscular behaviors of SMA worms: the pumping action that allows the worms to move food through their digestive tracts and the body bending that they perform when swimming.

To learn how Riluzole had this effect, they performed further experiments testing various potassium channels, including SK2, that Riluzole is known to act upon. Losing these channels didn’t cause more problems in animals with less SMN protein, but losing the SK2 potassium channels in particular made neuromuscular function worse. Without the SK2 channels, the drug Riluzole didn’t improve function.

“This told us that Riluzole improves motorneuron function by acting through SK2 channels, which we did not know before,” said lead author Maria Dimitriadi, a postdoctoral researcher in Hart’s group. “This is important bec

(via thescienceofreality)

futurejournalismproject:

White Men, Everyone Else: Gender and Ethnic Diversity on Cable News

Media Matters spent the month of April reviewing evening guests on cable news. The results, unfortunately, don’t surprise: CNN, Fox News, and MSNBC “overwhelmingly host male and white guests.”

Read through for the details as the watchdog group breaks down the numbers for each network. We learn, for instance, that “Out of 1,677 total guests, CNN had the largest proportion of men — 76 percent — during the month of April;” and “Fox News had the largest proportion of white guests — 83 percent.”

Hat tip to Chris Hayes, whose show is the most diverse in cable evening news. And getting there isn’t very difficult. “We just would look at the board and say, ‘We already have too many white men. We can’t have more,’” Hayes told Ann Friedman at the Columbia Journalism Review back in March. “Really, that was it.”

Images: Diversity On Evening Cable News, via Media Matters. Select to embiggen.

(via reallyfoxnews)

Skepticism About the Census Voter Turnout Finding

pewresearch:

The Census Bureau made big news last week when it reported that the black voter turnout rate (66.2%) exceeded the white voter turnout rate (64.1%) for the first time ever in 2012. But a closer look at the numbers raises some intriguing questions.

ilovecharts: This map shows unregistered user edits to Wikipedia articles in real time. Unregistered users account for only about 15% of English language Wikipedia edits, so this only represents a small portion of the total edits being made to Wikipedia. That being said, it is still wicked cool.

ilovecharts:

This map shows unregistered user edits to Wikipedia articles in real time. Unregistered users account for only about 15% of English language Wikipedia edits, so this only represents a small portion of the total edits being made to Wikipedia. That being said, it is still wicked cool.

pewresearch: The U.S. receives overwhelmingly positive ratings in Israel, with even more Israelis now saying they have a favorable view of their country’s ally than did so two years ago, when Pew Research last conducted a survey in Israel; today, 83% express a positive opinion of the U.S., compared with 72% in 2011. In contrast, about eight-in-ten (79%) Palestinians express unfavorable views of the U.S., virtually unchanged from recent surveys. Read more.

pewresearch:

The U.S. receives overwhelmingly positive ratings in Israel, with even more Israelis now saying they have a favorable view of their country’s ally than did so two years ago, when Pew Research last conducted a survey in Israel; today, 83% express a positive opinion of the U.S., compared with 72% in 2011. In contrast, about eight-in-ten (79%) Palestinians express unfavorable views of the U.S., virtually unchanged from recent surveys. Read more.

unhistorical:

May 15, 1536: Anne Boleyn is found guilty of treason.

Anne Boleyn, Henry VIII’s second wife after Catharine of Aragon and the wife for whom the king broke away from the Catholic Church, was arrested in May of 1536 and charged with adultery, incest, and treason. Her arrest took place only three years after her marriage to Henry, which had so far produced no male heirs and only one healthy child; the king had meanwhile taken Jane Seymour, who was to become his third wife just weeks after Anne Boleyn’s execution, as a mistress. Anne was, according to contemporary accounts, intelligent, witty, and anything but submissive. all traits that Henry found desirable, even exciting, in a mistress, but not in a wife; her confrontational nature combined with her failure to bear male heirs healthy enough to survive past infancy caused their marriage to crumble.

Anne Boleyn’s arrest was based on accusations of her illicit sexual relationships with a court musician, several aristocrats, and Anne’s own brother George; she was charged with both adultery (a form of treason when committed by a queen) and plotting the death of the king (another form of treason). Of her accused lovers, five were found guilty of treason, including George Boleyn, and executed by decapitation on May 17, 1536. Anne was held in the Tower of London and remained there until her own execution on May 19, 1536; her final words were reportedly a prayer:

To Jesus Christ I commend my soul; Lord Jesus receive my soul.

Anne Boleyn was survived by one child, who was the only one of her siblings to survive birth and infancy, who was declared illegitimate and deprived of her birthright not long after her mother’s execution in order to clear the way for her father’s male heirs, and who eventually became one of England’s most famous, most influential monarchs.