“It could be possible to reverse the muscle damage seen in children with a form of motor neurone disease,” according to BBC News. The condition in question – spinal muscular atrophy (SMA – causes deterioration of specific nerves and muscles in the body, and is sometimes known as ‘floppy baby syndrome’ due to the weakness it creates in the limbs. The condition reportedly affects 1 in 6,000 babies, with around half of children with the most severe form of this disease dying before the age of two.
Research has already established that the condition affects the nerves, and it has previously been thought that the muscles waste mainly as a result of this nerve damage. However, this news is based on an experiment in mice that suggests the muscles start to undergo changes even before the nerves deteriorate. Crucially, some of these changes could be reversed using a drug called SAHA, which has also been found to increase lifespan in SMA mice in a previous study.
The drug SAHA is already approved by the US Food and Drug Administration for use in a very specific form of cancer. The fact that this drug has already been tried in humans for another condition may make it easier to test in people with SMA. Trials will still need to be carried out before we can say whether this drug is effective and safe in humans. Drug treatments for this condition would be valuable, as there is currently no cure.
Where did the story come from?
BBC News’ coverage referred to two related papers from the same group of researchers based primarily at the University of Edinburgh. One of these research papers looked at the effect of using drugs called HDAC inhibitors on a mouse model of SMA, while the other paper focused only on the biology of a mouse model of SMA, but did not assess the effects of any treatments. This Behind the Headlines appraisal focuses on the former of these papers as online news reports tended to focus on the possibility of developing new treatments.
This HDAC inhibitor study was carried out by researchers from the University of Edinburgh, and other research centres in the UK and Germany. It was funded by SMA Trust, BDF Newlife, the Anatomical Society and Germany’s DFG research fund.
The study was published in the peer-reviewed medical journal Human Molecular Genetics.
The report from BBC News gives a very brief summary of the two studies, and provides more information about the condition SMA itself. It does report the fact that the treatment study was in mice.
What kind of research was this?
Spinal muscular atrophy (SMA is a form of motor neurone disease that is caused by mutations in the SMN1 gene, leading to degeneration of a type of nerve cell found in the spinal cord. These nerve cells, called motor neurones, normally carry messages from the brain to the muscles. In SMA, motor neurone degeneration causes progressive weakness of the limbs and trunk, followed by muscle wasting. About 1 in 6,000 to 1 in 10,000 babies are thought to be affected. Some forms of SMA typically lead to death in the first few years of life, and the condition is reportedly among the most common genetic causes of infant death. Other forms become apparent later in life and are less severe.
SMA is an ‘autosomal recessive’ disease, meaning that it only becomes apparent if an individual has two copies of a faulty gene, one inherited from each parent. People with only one faulty copy of the gene will not have the condition but are known as carriers, and can have a child with the condition if their partner is also a carrier. People with the condition have low levels of a protein called SMN.
This was animal research that looked at the changes that occur in the muscles of a mouse model of spinal muscular atrophy (SMA . It also looked at whether the changes could be reversed by a specific type of drug called a histone deacetylase (HDAC inhibitor.
The researchers say that, thus far, most research has looked at how this disease affects the nerves that send messages to the muscles, rather than the muscles themselves. They wanted to look at the effect of the disease on the muscle in mouse models of SMA.
Animal models are very useful for studying aspects of the biology of human diseases that would be hard to study in humans. They are also essential for the initial testing of drugs that might be useful for treating human conditions, to make sure they are safe and effective enough to test in humans. These animal tests should be seen as only the first of many stages, as drugs that show promise in these tests are not always effective or safe in humans, due to differences between the species.
What did the research involve?
In their first set of experiments the researchers used a mouse model of SMA that causes a severe form of the condition.
In SMA the nerves that send signals to the muscles break down, and this then leads to loss of muscle fibres. The researchers looked specifically at one muscle that does not lose its nerve signals early in the disease, so they could see whether any changes in the muscle happen independently of the problems with the nerves.
The researchers looked at what changes happened to the proteins in this muscle before the mice developed any symptoms. They found that the proteins that were affected related to cell death, so they then looked at whether there were signs that more cells were dying in muscles of SMA mice than in muscles of normal mice. They also looked at whether some of the protein changes seen in mice were also seen in samples of muscle taken from human SMA patients.
Previous research has suggested that chemicals called HDAC inhibitors can increase levels of the SMN protein in mouse models of SMA, and reduce muscle loss. Based on this the researchers decided to test whether HDAC inhibitors directly affected the muscle. These experiments used a different mouse model that causes a less severe form of the condition. They say that this model is better for testing the effects of potential treatments for the disease because the mice live slightly longer.
The mice were given an HDAC inhibitor called suberoylanilide hydroxamic acid (SAHA orally from birth. Control mice were given no SAHA. The researchers looked at the effect of this treatment on the levels of the different proteins in the muscles that are affected by SMA. SAHA (Vorinostat is approved by the US Food and Drug Administration for use as a treatment for a specific type of cancer in humans.
What were the basic results?
The researchers found that the muscle from pre-symptomatic SMA mice showed differences in the levels of a number of proteins compared with normal mice. This was despite the fact that the nerves sending messages to the muscle were not yet affected. This finding suggests that the condition starts to affect the muscle even before any deterioration occurs as a result of changes in the nerves.
Many of the proteins affected by the condition were found to be involved in muscle function or cell death. The researchers found that there were also other signs of increased cell death in the muscle of SMA mice compared with normal mice.
The researchers then examined human SMA muscle to look at two proteins found at abnormal levels in SMA mouse tissue: one protein called Vdac2 that was found at higher levels in the SMA mouse muscle and one protein called parvalbumin that was found at lower levels in the SMA mouse muscle. They found that levels of these two proteins were similarly affected in human SMA muscle tissue.
Treating SMA mice with the HDAC inhibitor drug SAHA from birth increased levels of SMN protein in their muscle. SAHA treatment also reversed the changes seen in the levels of the proteins Vdac2 and parvalbumin, although the levels of parvalbumin were still not quite as high as in normal muscle. SAHA treatment also reduced the levels of a protein called H2AX, which is involved in cell death and which was significantly raised in SMA mice.
How did the researchers interpret the results?
The researchers concluded that the molecular effects of SMA on muscle in mice were improved by the existing FDA-approved drug SAHA. They say that their study showed that abnormalities in skeletal muscle tissue proteins are an important and potentially reversible part of SMA.
Conclusion
This study has shown that in mouse models of spinal muscular atrophy (SMA , before problems develop in the nerves that send messages to the muscles, the muscles themselves have abnormal levels of certain proteins. Human SMA muscle tissue was also found to have some of these abnormalities. Notably, the researchers also showed that, in mice, some of these abnormalities could be reversed using a drug called SAHA, which belongs to a group of drugs called the HDAC inhibitors.
Previous studies have suggested that SAHA treatment increased the lifespan of mice with SMA. The current study did not look at the effect of this drug on symptoms or lifespan in these mice, just at its effect on particular proteins within the muscle.
The drug SAHA is already approved by the US Food and Drug Administration for use in a very specific form of cancer (cutaneous manifestations of cutaneous T-cell lymphoma . The drug does not appear to have been approved for use in Europe for this type of cancer or for other conditions. The fact that this drug has already been tried in humans for another condition may make it easier to test this drug in people with SMA. Such trials will need to be carried out before we can say whether this drug is effective and safe for the treatment of SMA. New treatments for this condition would be valuable, as there is currently no cure.
Analysis by Bazian
Links To The Headlines
Child motor neurone disease treatment clue. BBC News, February 27 2012
Links To Science
Mutsaers CA, Wishart TM, Lamont DJ et al. Reversible molecular pathology of skeletal muscle in spinal muscular atrophy. Human Molecular Genetics (2011 20 (22 : 4334-4344
With that in mind, they attended a February 21 Town Hall meeting in Burlington, WA -- one of 10 Town Halls hosted by
United Fresh across the nation this month and next.
They didn't go home disappointed.
"There's a lot of new information to learn," Karen said after the meeting. "New things are happening all of the time."
New things like how the
Food Safety Modernization Act will affect the produce industry; what opportunities the government's push for more fruit and vegetables in school lunch and breakfast programs will offer growers; what's up with the 2012 Farm Bill; improved ways to track produce from the farm to the buyer; and even smart phones that can scan bar codes and give consumers information about the farmer who produced the food they're buying.
On the food-safety front, the Fuentes said they're working toward
Good Agricultural Practices certification, which is designed to establish food-safety practices on the farm.
"It might sound overwhelming at first," Karen said, referring to GAP certification, "but not if you think of it as a step-by-step process."
Their interest in food safety mirrors United Fresh's interest in food safety. It was the first topic on the docket during the Town Hall session the couple attended.
"Top of the mind" for the produce industry is how Robert Guenther, senior vice president of Public Policy for United Fresh, described the
Food Safety Modernization Act, signed into law by President Obama last year. "It's probably the most significant law to change our industry," Guenther said.
To be implemented over 3 years, the law, which calls for 12 new U.S. Food and Drug Administration regulations, gives the FDA additional responsibilities, among them enhanced inspections, recordkeeping and reporting requirements, traceback pilot and training programs, and mandatory recall authority.
But underlying the act itself is a fundamental shift in food-safety policy: The focus will be on prevention instead of on responding to outbreaks.
For growers and processors, it comes down to meeting science-based standards.
But the new regs won't be a top-down set of rules, according to Guenther. Instead, there will also be significant amount of collaboration with states and various local authorities.
What about the proposed rules?
Although the law said that the FDA needed to release a set of proposed rules, one of them dealing with produce, by Jan. 4, that date came and went without any sign of the proposed rules.
However, drafts of the proposed rules have been sent to the Office of Management and Budget. Guenther said that the proposed rules will likely be released in the Federal Register in late March.
No one in the produce industry has seen the drafts.
Guenther predicts that considering the procedures that must be followed, including public comment periods that last 90 to 120 days, before the final rules are adopted, it will probably be 2013 before that happens, instead of July 2012 as originally expected.
What about the produce rule?
Guenther told the group that the produce industry has a lot of questions about what the final produce rule will look like. For example, will it spell out requirements for specific fruits and vegetables or will it be a sweeping practice-based rule that covers all produce?
As for Congress's role in all of this, Guenther said that in the current economic climate, elected officials are not eager to pass new rules that impact businesses.
"They be watching this closely," he said. "It (budget woes could slow this down during the election year."
Healthier food for kids
Guenther praised the new
"school-meal rule" as a "bright spot" for the industry because the nutrition standards for the National School Lunch and Breakfast Program double the amount of fruits and vegetables school children will be served in their lunches. In addition, a doubling of fruits served in the breakfast program will be phased in over the next 3 years.
"It's a big victory for our industry," he said.
Even so, as produce-industry reps often point out, schools will be very vigilant about making sure the growers and processors they buy from are following strict food-safety practices.
2012 Farm Bill
Until 2008, the produce industry regarded the
Farm Bill as legislation geared to benefit subsidized commodity crops such as wheat, rice and corn and therefore of little interest to the produce industry. But Guenther said that in the mid-2000s, the industry started looking at the Farm Bill as a possible opportunity.
The question that provoked that new interest came down to this: "Why is 50 percent of plant-based agriculture not included in the farm bills?"
Thanks to a unified effort on the part of many agriculture associations across the country, the 2008 Farm Bill contained what Guenther described as "unprecedented funding" for specialty crops. (Examples of speciality crops are fruits and vegetables.
The $3 billion in funding included money for state block grants, targeted research programs, and increased access for fresh produce in federal nutrition programs.
Guenther said the produce industry benefited from this additional funding all the way through the distribution chain, from the farm to the retail level. And he pointed to food safety as a good example of how these different sectors of the industry benefited from the funding.
But with ongoing budget woes, coupled with the fact that the nation currently has a strong farm economy, there are questions about how the specialty crops will fare in the 2012 Farm Bill -- or even if there will be a Farm Bill this year.
"Election year politics have divided committees on this," Guenther said, referring to the Farm Bill.
Another problem for the produce industry is that research funding through the previous Farm Bill stops this year.
Tracking the produce
"We want consumers to always feel comfortable that what they're going to buy or consume is safe," said Dan Vache, vice president of Supply Chain Management for United Fresh, in describing the reason why traceability is so important.
The 2006 E. coli outbreak from fresh cut spinach, which caused 276 illnesses and 3 deaths -- not to mention huge financial losses for spinach growers and packers -- was a wake-up call for the industry. Once awakened, many in the industry joined together to launch the Produce Traceability Initiative in 2007. The goal was to take away some of the questions there might be about where a product was grown, packed, distributed, and even who handled it.
Vache said that even though the 2006 outbreak was caused by spinach in just one location in California's Salinas Valley, "it caused havoc for our industry overall."
Fast forward to today and the FDA wants electronic records, a common language (known as GS1 , and quick information.
"They want it within 24 hours; we're finding that sometimes we can do it in a matter of hours," said Vache.
Milestones in the initiative's progress include a 14-digit number that shows, by lot number, who you are and what products are being shipped. In addition, information about who "touched" the product throughout the distribution chain can be accessed. For example, in the case of inbound shipments, a bar code stores information about that.
This year, the goal is to be able to trace outbound cases of food.
Citing last year's cantaloupe Listeria outbreak, which killed more than 30 people, as an example of why outbound information is important, Vache said that no one was tracking where the contaminated cantaloupes grown in Colorado were being shipped.
He said that the lack of consumer confidence in cantaloupes due to the Listeria outbreak had growers in other states plowing under hundreds of acres of perfectly good cantaloupes.
Vache referred to that reaction on the part of the consumers as "consumer psyche."
"It's all about consumer confidence," Vache said, referring to traceability.
Shipping produce
For a produce grower or broker, there's nothing worse than having the produce, which was in excellent condition when it was sent on its way, show up at its final destination in poor shape. That can happen if the produce wasn't handled properly every step of the way during shipping.
With that in mind, the
North American Produce Transportation Working Group, which is made up of volunteers from more than 25 national and regional produce industry associations, transportation providers, grower/shippers and perishable receivers, has crafted a set of best practices pertaining to the handling and transportation of fresh produce.
These best practices, which can be updated should changes in the industry arise, were developed to identify problem areas in the marketing chain where increased communication and agreed-upon procedures can prevent many issues in the marketing chain before they result in a problem at the final destination.
The goal is to prevent disputes.
What about small-scale producers?
In an interview directly after the Town Hall meeting in Burlington, WA, Vache and Guenther said that small-scale growers need to recognize that the world has changed.
Pointing out that the final rules in the Food Safety Modernization Act will serve as a "framework for safe practices," Guenther said these food-safety practices will be demanded by most customers.
"It will be important for growers to be able to explain to consumers that their food is safe," Guenther said. "They'll need to have a good message about what they're doing on the farm. It's very important for them to use current tools to get up to date."
As for bugs (foodborne pathogens such as E. coli, Salmonella and Listeria that can sicken or even kill people , Guenther said the bugs don't know if they're on a big or small farm.
"If you're selling to consumers, you need safeguards to protect your crops from potential contamination," he said.
Town Hall meetings
Go here for more information about United Fresh's Town Hall meetings, its 2012 Fresh Impact Tour, and its 2012 annual convention in Dallas, TX.
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Top photo of Robert Guenther, lower photo of Dan Vache
message for this year's Lent, calling on Catholics to "Let us be concerned for each other, to stir a response in love and good works," it seems the Church hierarchy in the United States has a different take: screw you, in the name of the Lord.
Still outraged—outraged!—that the majority of Americans support insurance coverage of contraception, used by almost all sexually active women, including Catholics, the bishops and cardinals aren't shying away from its absurd rhetoric and threats.
Via Joe My God, Cardinal Francis George of the Archdiocese of Chicago, has penned a
lengthy column suggesting that Church-affiliated hospitals should shut down and cease providing health care services altogether, rather than be forced to ... uh ... exist in a country in which women have access to birth control:
What will happen if the HHS regulations are not rescinded? A Catholic institution, so far as I can see right now, will have one of four choices: 1 secularize itself, breaking its connection to the church, her moral and social teachings and the oversight of its ministry by the local bishop. This is a form of theft. It means the church will not be permitted to have an institutional voice in public life. 2 Pay exorbitant annual fines to avoid paying for insurance policies that cover abortifacient drugs, artificial contraception and sterilization. This is not economically sustainable. 3 Sell the institution to a non-Catholic group or to a local government. 4 Close down.Of course, none of those things are actually true, since the new policy adopted by the Obama administration doesn't force the Church to do a damn thing, won't cost the Church a dime, doesn't cover abortifacient drugs, and
is
, in fact, not only "economically sustainable," but far more cost-efficient than denying contraception to women. In other words, the cardinal is flat-out lying about what the policy does and doesn't do. Hey, isn't there
something in the Bible about how you're not supposed to make shit up?
The cardinal acknowledges that "some" (actually, Father, it's just about
all
Catholics don't actually subscribe to the no-contraception rule, he's got news for them:
There have always been those whose personal faith is not adequate to the faith of the church. Perhaps this is the time for everyone to re-read the Acts of the Apostles. Bishops are the successors of the apostles; they collectively receive the authority to teach and govern that Christ bestowed upon the apostles. Bishops don’t claim to speak for every baptized Catholic. Bishops speak, rather, for the Catholic and apostolic faith. Those who hold that faith gather with them; others go their own way. They are and should be free to do so, but they deceive themselves and others in calling their organizations Catholic.Riiiiiight. So the 98 percent of sexually active Catholic women who've relied on some form of contraception are not adequately Catholic. Bunch of fakers. Because the only way to be adequately Catholic is to do exactly what the bishops command. It's right there in the Bible!
Bishops are the boss of you, and if you don't do what they say, you aren't a good Catholic, sayeth the Lord.
So having suggested that the Church should deny all health care to all people rather than stand by while its laity continues to use contraception just as it always has, and having chastised that laity for being inadequately subservient to the Church hierarchy, the cardinal concludes:
I ask lay Catholics and others of good will to step back and understand what is happening to our country as the church is despoiled of her institutions and as freedom of conscience and of religion become a memory from a happier past. The suffering being imposed on the church and on society now is not a voluntary penance.Ah yes, the Church and society are indeed suffering by being forced to ... wait, what is it the Church is being forced to do? Oh, right. Absolutely nothing.
But apparently that's reason enough to threaten to shut down hospitals and deny care of any kind to anyone in need. In the name of the Lord.
Of course, the Church has already lost this battle. And it knows that; thus, the increasingly desperate complaints and threats that prove the "concern for each other" and acts of "love and good works" don't mean nearly as much to the Church hierarchy as demanding that the government enforce its antiquated edicts. Because lord knows its own "inadequate" laity has stopped listening.
The coalition explained
in a letter to FDA Commissioner Margaret Hamburg that more consumers are trying to make better, informed choices about the foods they eat and that FDA has the ability to provide consumers with the informational tools to make smarter decisions.
The letter noted that "[w]hile current regulations stipulate what foods can be labeled 'No Sugar Added' or use a similar phrase, there is currently no requirement that added sugars be shown separately on the 'Ingredients List.' "
The coalition recommends that FDA require any "added sugars" to be listed in the ingredients section as a single food ingredient with a parenthetical list of the specific ingredients that account for those sugars. In line with the rules for labeling ingredients, the specific added sugars would be listed by descending weight and the combined weight of the added sugars will be used to determine where the "added sugars" ingredient will be ranked in the ingredient list.
Americans consume significantly more added sugar than is recommended. The American Heart Association, which signed the letter, notes that the usual intake of added sugars for Americans is 22.2 teaspoons a day. At first glance that may not sound like a lot of added sugar, but 22.2 teaspoons of sugar is the equivalent of 355 calories; the recommended daily limit of added sugars for women is 100 calories and 150 for men.
The connection between sugar intake and the obesity epidemic is also of concern to this coalition. According to the Center for Disease Control and Prevention, more than 33 percent of adults and approximately 17 percent of children and adolescents are obese.
Likely anticipating a response from the sugar and food industry that the burden should fall on the consumer to stay informed to make these decisions rather than impose more government regulation, the coalition stated: "Without specific information on the amount of 'added sugars' on the labels of food products, consumers can hardly exercise that responsibility and make smarter choices in the grocery aisle."
The 14 coalition members are: Environmental Working Group, American Association for Health Education, American Heart Association, Center for Science in the Public Interest, Corporate Accountability International, Defeat Diabetes Foundation, American Association for Health Education, National Association of School Nurses, Young People's Healthy Heart at Mercy Hospital, Indiana Rural Health Association, American Society of Bariatric Physicians, The FGE Food & Nutrition Team, Cambridge/Somerville WIC, and Iowa Public Health Association.
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