New knowledge about serious muscle disease

PUBLIC RELEASE DATE:

31-Oct-2013

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Contact: Malene Bech Vester-Christensen
mbxc@novonordisk.com
45-22-15-25-89
University of Copenhagen

About 3,000 people in Denmark suffer from one of the serious muscle-related diseases that come under the heading of muscular dystrophy. Some patients diagnosed with muscular dystrophy die shortly after birth, others become severely retarded and develop eye problems, while certain groups are confined to life in a wheelchair. Common to all muscular dystrophy sufferers is the difficulty of their muscle cells to attach themselves to each other and to the surrounding tissue. However, little is actually known about the root causes of the disease.

New basic research from University of Copenhagen now offers insight into previously unknown facts about muscular dystrophy that may improve future diagnosis and treatment of the disease. The findings have just been published in the scientific journal PNAS (Proceedings of the National Academy of Science).

"Our new research findings may shed light on some of the cellular processes that take place in connection with, for example, muscular dystrophy. This is important information because it is crucial for us to gain as detailed an understanding as possible about the individual cell components. Although the journey from the current basic research to any potential treatment options or diagnostic tools is a long one, our discoveries give grounds for optimism," says postdoc Malene Bech Vester-Christensen who carried out the new experiments from her base at the Faculty of Health and Medical Sciences, University of Copenhagen, and has since taken up a research position at Novo Nordisk.

Sugar molecules central to our organism

The new method developed by researchers makes it easier to map the proteins that The protein previously associated with muscular dystrophy is a so-called glycoprotein a protein with chains of sugar molecules attached. The special kind of sugar attached to these glycoproteins is called mannose. A functional pathway for binding mannose to the proteins is key to the functioning of the human organism, and genetic defects in the process that attaches mannose to the proteins known as O-mannosylation can lead to diseases such as muscular dystrophy.

"To date, only one single protein has been identified and characterised where the mannose deficiency on the protein leads to muscular dystrophy, but our method enables us to faster identify many new proteins that have mannose attached and therefore potentially play a key role for the disease," says Adnan Halim, who is associated with the research project and a postdoc with the Danish National Research Foundation, Copenhagen Center for Glycomics.

Facts about muscular dystrophy

Muscular dystrophy is a collective term for a range of neuromuscular disorders. There are roughly 100 different known muscular dystrophy diagnoses, which manifest themselves as various functional impairments/disabilities. The individual diagnoses vary greatly, as does the manner in which they develop. Some people experience only a few symptoms, while others suffer extensive functional impairment. About 3,000 people in Denmark suffer from one of the muscle-related diseases classified as muscular dystrophy. Muscular dystrophy cannot be cured, but much can be done to relieve and treat the consequences of the diseases. Source: The Danish Muscular Dystrophy Foundation.

Facts about the research

The researchers used cells with a functional pathway to attach mannose to proteins, but then simplified the proteins' sugar chains by removing the gene responsible for lengthening the chains and making them more complex. The proteins carrying the 'sugar chain', which now consists solely of mannose, were then isolated, enabling the researchers to determine which proteins carried mannose and where the mannose was situated.

Facts about mannose

For many years researchers believed that O-linked mannose on proteins was found only in yeast, but recent studies have showed that the mannose sugar molecule also binds to some of the proteins in human cells. Mannose plays a key role in the binding of muscle cells in tissue, and the lack of mannose on certain proteins can lead to muscular dystrophy. The variations in the degree of severity of the defects in the process known as O-mannosylation that attaches mannose to proteins and probably also which proteins carry mannose are key in determining how the disease manifests itself and its severity.

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PUBLIC RELEASE DATE:

31-Oct-2013

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Contact: Malene Bech Vester-Christensen
mbxc@novonordisk.com
45-22-15-25-89
University of Copenhagen

About 3,000 people in Denmark suffer from one of the serious muscle-related diseases that come under the heading of muscular dystrophy. Some patients diagnosed with muscular dystrophy die shortly after birth, others become severely retarded and develop eye problems, while certain groups are confined to life in a wheelchair. Common to all muscular dystrophy sufferers is the difficulty of their muscle cells to attach themselves to each other and to the surrounding tissue. However, little is actually known about the root causes of the disease.

New basic research from University of Copenhagen now offers insight into previously unknown facts about muscular dystrophy that may improve future diagnosis and treatment of the disease. The findings have just been published in the scientific journal PNAS (Proceedings of the National Academy of Science).

"Our new research findings may shed light on some of the cellular processes that take place in connection with, for example, muscular dystrophy. This is important information because it is crucial for us to gain as detailed an understanding as possible about the individual cell components. Although the journey from the current basic research to any potential treatment options or diagnostic tools is a long one, our discoveries give grounds for optimism," says postdoc Malene Bech Vester-Christensen who carried out the new experiments from her base at the Faculty of Health and Medical Sciences, University of Copenhagen, and has since taken up a research position at Novo Nordisk.

Sugar molecules central to our organism

The new method developed by researchers makes it easier to map the proteins that The protein previously associated with muscular dystrophy is a so-called glycoprotein a protein with chains of sugar molecules attached. The special kind of sugar attached to these glycoproteins is called mannose. A functional pathway for binding mannose to the proteins is key to the functioning of the human organism, and genetic defects in the process that attaches mannose to the proteins known as O-mannosylation can lead to diseases such as muscular dystrophy.

"To date, only one single protein has been identified and characterised where the mannose deficiency on the protein leads to muscular dystrophy, but our method enables us to faster identify many new proteins that have mannose attached and therefore potentially play a key role for the disease," says Adnan Halim, who is associated with the research project and a postdoc with the Danish National Research Foundation, Copenhagen Center for Glycomics.

Facts about muscular dystrophy

Muscular dystrophy is a collective term for a range of neuromuscular disorders. There are roughly 100 different known muscular dystrophy diagnoses, which manifest themselves as various functional impairments/disabilities. The individual diagnoses vary greatly, as does the manner in which they develop. Some people experience only a few symptoms, while others suffer extensive functional impairment. About 3,000 people in Denmark suffer from one of the muscle-related diseases classified as muscular dystrophy. Muscular dystrophy cannot be cured, but much can be done to relieve and treat the consequences of the diseases. Source: The Danish Muscular Dystrophy Foundation.

Facts about the research

The researchers used cells with a functional pathway to attach mannose to proteins, but then simplified the proteins' sugar chains by removing the gene responsible for lengthening the chains and making them more complex. The proteins carrying the 'sugar chain', which now consists solely of mannose, were then isolated, enabling the researchers to determine which proteins carried mannose and where the mannose was situated.

Facts about mannose

For many years researchers believed that O-linked mannose on proteins was found only in yeast, but recent studies have showed that the mannose sugar molecule also binds to some of the proteins in human cells. Mannose plays a key role in the binding of muscle cells in tissue, and the lack of mannose on certain proteins can lead to muscular dystrophy. The variations in the degree of severity of the defects in the process known as O-mannosylation that attaches mannose to proteins and probably also which proteins carry mannose are key in determining how the disease manifests itself and its severity.

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Source: http://www.eurekalert.org/pub_releases/2013-10/uoc-nka103113.php
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Texas club auctions right to hunt endangered rhino

In this Jan. 5, 2003, photo released by U.S. Fish and Wildlife Service shows a black rhino male and calf in Mkuze, South Africa. The organizer of a Texas hunting club’s planned auction of a permit that will allow a hunter to bag an endangered black rhino in Africa is hoping it raises up to $1 million for rhino preservation. (AP Photo/U.S. Fish and Wildlife Service, Karl Stromayer)

In this Jan. 5, 2003, photo released by U.S. Fish and Wildlife Service shows a black rhino male and calf in Mkuze, South Africa. The organizer of a Texas hunting club’s planned auction of a permit that will allow a hunter to bag an endangered black rhino in Africa is hoping it raises up to $1 million for rhino preservation. (AP Photo/U.S. Fish and Wildlife Service, Karl Stromayer)

HOUSTON (AP) — Plans to auction a rare permit that will allow a hunter to take down an endangered black rhino are drawing criticism from some conservationists, but the organizer says the fundraiser could bring in more than $1 million that would go toward protecting the species.

John J. Jackson III belongs to the Dallas Safari Club, which earlier this month announced it would auction the permit — one of only five offered annually by Namibia in southwestern Africa. The permit is also the first to be made available for purchase outside of that country.

"This is advanced, state-of-the-art wildlife conservation and management techniques," Jackson, a Metairie, La.-based international wildlife attorney, said Wednesday. "It's not something the layman understands, but they should.

"This is the most sophisticated management strategy devised," he said. "The conservation hunt is a hero in the hunting community."

Some animal preservation groups are bashing the idea.

"More than ridiculous," Wayne Pacelle, president of the Humane Society of the United States, said Wednesday.

"At a time when the global community is rallying to protect the elephant and rhino from the onslaught of people with high-powered weapons, this action sends exactly the wrong signal. It's absurd. You're going to help an endangered animal by killing an endangered member of that population?"

An estimated 4,000 black rhinos remain in the wild, down from 70,000 in the 1960s. Nearly 1,800 are in Namibia, according to the safari club.

Poachers long have targeted all species of rhino, primarily for its horn, which is valuable on the international black market. Made of the protein keratin, the chief component in fingernails and hooves, the horn has been used in carvings and for medicinal purposes, mostly in Asia. The near extinction of the species also has been attributed to habitat loss.

The auction is scheduled for the Dallas Safari Club's annual convention in January.

According to Jackson, who said he's been working on the auction project with federal wildlife officials, the hunt will involve one of five black rhinos selected by a committee and approved by the Namibian government. The five are to be older males, incapable of reproducing and likely "troublemakers ... bad guys that are killing other rhinos," he said.

"You end up eliminating that rhino and you actually increase the reproduction of the population."

Jackson said 100 percent of the auction proceeds would go to a trust fund, be held there until the permit is approved and then forwarded to the government of Namibia for the limited purpose of rhino conservation.

"It's going to generate a sum of money large enough to be enormously meaningful in Namibia's fight to ensure the future of its black rhino populations," Ben Carter, the club's executive director, said in a statement.

Jeffrey Flocken, North American regional director of the Massachusetts-based International Fund for Animal Welfare, disagreed, describing the club's argument as "perverse, to say the least."

"And drumming up a bidding frenzy to get to the opportunity to shoot one of the last of a species is just irresponsible," Flocken said. "This is just an attempt to manipulate a horrific situation where rhino poaching is out of control, and fuel excitement around being able to kill an animal whose future existence is already hanging in the balance."

Rick Barongi, director of the Houston Zoo and vice president of the International Rhino Foundation, said the hunt was not illegal but remained a complex idea that "sends a mixed message."

On Tuesday, the U.S. Fish and Wildlife Service announced it was providing "guidance" to the safari club on whether it would agree to a permit, required under federal law, to allow the winning bidder to bring the trophy rhino to the United States.

"An import permit will be issued if, and only if, we determine that the sport-hunted trophy is taken as part of a well-managed conservation program that enhances the long-term survival of the species," the agency said.

Earlier this year, the service granted such a permit for a sport-hunted black rhino taken in Namibia in 2009.

Pacelle said the Humane Society would work to oppose the permit.

An administrator at the Namibian Embassy in Washington referred questions about the hunt and auction to the government's tourism office in Windhoek, the nation's capital. There was no response Wednesday to an email from The Associated Press.

"The two hot issues here are the fact it's an endangered species, and the second thing is it's a trophy," Barongi, the zoo director, said. "It's one individual that can save hundreds of individuals, and if that's the case, and it's the best option you have ... then you go with your best option.

"Because the alternative is you can lose them all," he said.

Associated PressSource: http://hosted2.ap.org/APDEFAULT/b2f0ca3a594644ee9e50a8ec4ce2d6de/Article_2013-10-31-US-Rhino-Hunt-Auction/id-c3e2992e22e14aa5928855e6c44eefdc
Tags: marine corps marathon   raiders   Tom Clancy   oakland raiders   rosh hashanah  

Risk of osteoporosis drug's side effects not significant, Loyola researchers find

PUBLIC RELEASE DATE:

30-Oct-2013

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Contact: Nora Dudley
nodudley@lumc.edu
708-216-6268
Loyola University Health System

The risks of developing kidney failure and a calcium deficiency from the popular osteoporosis drug zoledronic acid are extremely rare, according to researchers at Loyola University Health System (LUHS). These findings were presented earlier this month at the American Society for Bone and Mineral Research's annual meeting.

"Osteoporosis is a growing problem in this country," said Laurae Hicks, first author and Stritch School of Medicine medical student. "These findings are valuable for the millions of Americans who suffer from osteoporosis and are considering their treatment options."

Zoledronic acid is commonly used to treat osteoporosis. The treatment strengthens bones by increasing the process by which bone is broken down and replaced with new bone tissue. While this medication is effective at preventing and treating osteoporosis, potential side effects include kidney failure and hypocalcemia.

Kidney failure occurs when the kidneys fail to adequately filter waste products from the blood while hypocalcemia is characterized by low calcium levels in the blood. This condition can lead to a variety of symptoms, including weakness, muscle cramps, excessive nervousness, headaches or uncontrollable twitching and cramping in certain muscles.

"This study helped us determine the severity and prevalence of these side effects," said Pauline Camacho, MD, study investigator and director of the Loyola University Osteoporosis and Metabolic Bone Disease Center. "This will help us assess which patients are good candidates for this treatment."

Researchers studied 237 patients before and after they received injections of zoledronic acid. They found that a slight and clinically insignificant decline in calcium levels may be seen after the first infusion, but these effects appear to be transient. The findings apply only to individuals with normal vitamin D levels and kidney function prior to infusion and cannot be generalized to those with renal insufficiency and existing calcium and vitamin D deficiencies.

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PUBLIC RELEASE DATE:

30-Oct-2013

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Contact: Nora Dudley
nodudley@lumc.edu
708-216-6268
Loyola University Health System

The risks of developing kidney failure and a calcium deficiency from the popular osteoporosis drug zoledronic acid are extremely rare, according to researchers at Loyola University Health System (LUHS). These findings were presented earlier this month at the American Society for Bone and Mineral Research's annual meeting.

"Osteoporosis is a growing problem in this country," said Laurae Hicks, first author and Stritch School of Medicine medical student. "These findings are valuable for the millions of Americans who suffer from osteoporosis and are considering their treatment options."

Zoledronic acid is commonly used to treat osteoporosis. The treatment strengthens bones by increasing the process by which bone is broken down and replaced with new bone tissue. While this medication is effective at preventing and treating osteoporosis, potential side effects include kidney failure and hypocalcemia.

Kidney failure occurs when the kidneys fail to adequately filter waste products from the blood while hypocalcemia is characterized by low calcium levels in the blood. This condition can lead to a variety of symptoms, including weakness, muscle cramps, excessive nervousness, headaches or uncontrollable twitching and cramping in certain muscles.

"This study helped us determine the severity and prevalence of these side effects," said Pauline Camacho, MD, study investigator and director of the Loyola University Osteoporosis and Metabolic Bone Disease Center. "This will help us assess which patients are good candidates for this treatment."

Researchers studied 237 patients before and after they received injections of zoledronic acid. They found that a slight and clinically insignificant decline in calcium levels may be seen after the first infusion, but these effects appear to be transient. The findings apply only to individuals with normal vitamin D levels and kidney function prior to infusion and cannot be generalized to those with renal insufficiency and existing calcium and vitamin D deficiencies.

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AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert! system.

Source: http://www.eurekalert.org/pub_releases/2013-10/luhs-roo103013.php
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Paris Hilton: "Twerk or Treat" at the Playboy Mansion

It's one of the biggest Halloween parties in town and Paris Hilton made sure to attend the annual bash at the Playboy Mansion on Saturday (October 26).

Dressing in what will likely be one of the most popular costumes this year, the blonde socialite sported a gray leotard with a bear on it and tiny pigtails as she posed with her tongue out in her best Miley Cyrus impression.

Sharing a photo of herself in costume in the limo before arrival, the 32-year-old star tweeted, "Twerk or Treat."

Just the day before, Paris headed to another costume party, making sure to debut a different look. On Friday, she tweeted a pic with the caption, "Thanks @ElectricLaundry for the beautiful Barbie costume you made me. And also thanks @EtienneOrtega for the fierce hair & makeup. Loves it."

Source: http://celebrity-gossip.net/paris-hilton/paris-hilton-twerk-or-treat-playboy-mansion-950726
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Celebrity-store partnerships run risks

NEW YORK (AP) — When big-name celebrities pair up with big businesses, customers often believe the adage: You are the company you keep.

Rap artist Jay-Z is learning that firsthand. He has complained this week that he's been unfairly "demonized" because he hasn't backed out of his collaboration with Barneys New York after the luxury retailer was accused of racially profiling two black customers.

Jay-Z, whose real name is Shawn Carter, has said he's waiting to hear all the facts. Meanwhile, Barneys said on Tuesday that its initial investigation showed no employees were at fault in the two incidents in which customers complained that they were detained by police after making expensive purchases. NYPD disputes the store's account and said they were alerted by Barneys.

The controversy illustrates the problems that can arise when celebrities and companies team up.

The deals are lucrative: Companies like having big names on their roster and celebrities are always looking to expand their brand. Revenue in North America from celebrity merchandise lines, excluding products linked to athletes, was a $7.8 billion business last year, according to figures available from trade publication Licensing Letter.

But when either side is accused of wrongdoing, the negative publicity can cause damage to their partner's reputation.

"It literally shows you how vulnerable the celebrity business is on both sides of the equation," said Marshal Cohen, chief retail analyst at The NPD Group, a market research group.

More often, it's the celebrities — not the stores — who are accused of bad behavior.

Late last year, for instance, Macy's was pressured by some customers to dump real estate mogul Donald Trump's line of $65 power ties after the billionaire verbally attacked President Barack Obama on social media following his re-election. One customer collected close to 700,000 signatures on a petition website signon.org. Macy's stood by Trump.

Another example: home maven Martha Stewart. After being convicted on federal criminal charges of lying to prosecutors about a stock sale, she served a five-month prison sentence that ended in 2005. Kmart, which sold her towels and kitchen accessories until 2009, continued to carry her line.

But experts say that the subject of race can stir up even more emotions, so there's less tolerance for slip-ups. "Everybody wants to be fair minded and not make generalizations about a group," said Marty Brochstein of the International Licensing Industry Merchandisers' Association, a trade group.

Celebrity chef Paula Deen's empire, which spanned from pots to TV shows, began to unravel in June, within days of the public disclosure of a legal deposition in she admitted under oath to having used the N-word to describe black employees. In addition to losing TV shows and book deals, Deen lost valuable partnerships when Target and other retailers said they'd no longer sell her products.

Until now, Michael Stone, CEO of brand licensing agency Beanstalk, says it's been the norm for personalities to have moral clauses in contracts that let merchants back out. But Stone, who has reviewed 100 celebrity contracts, says he hasn't seen it the other way.

For Jay-Z's part, it's not clear what he'll do as he faces pressure from an online petition and Twitter messages from fans. NYPD disputes the store's account of the incidents and said they were alerted by Barneys.

Barneys is expected to start selling items next month by top designers, inspired by Jay-Z, with some of the proceeds going to his charity. Jay-Z is also working with the store to create its artistic holiday window display.

• Society & Culture
• Barneys New York

Source: http://news.yahoo.com/celebrity-store-partnerships-run-risks-185403695--finance.html
Tags: carrie   tom hanks   emmy awards   Ariel Castro   Iggy Azalea  

Obamacare’s Newest Ills

In this file photo a mother and daughter wait to see their doctor in Dorchester, Mass. Americans continue to have problems using healthcare.gov to replace their insurance plans that will no longer be valid under Obamacare.

Photo by Joe Raedle/Getty Images

Obamacare is nerve-wracking for the very people it was intended to help. During the healthcare.gov saga, I've been in regular touch with Alice, a Pennsylvania woman trying to sign up for health insurance through the federal exchanges. She is a member of that 5 percent of the population who buys insurance in the individual market. When the Affordable Care Act took effect on Oct. 1, she was anxious to register for new health care coverage, because it is almost certain to be a better deal than her current plan. Instead, like millions of Americans, she has been confused and thwarted over the last month as government’s website failed her.

Her stress and confusion level recently quadrupled when her old insurance company, Independence Blue Cross, sent her a letter reminding her that Obamacare meant her old policy was expiring and that "to ensure continuous coverage, you'll need to select a new plan by December 15, 2013." 

If everything had gone as promised, by now she would have had a chance to sign up for insurance through the window-shopping wonders of healthcare.gov. Or, at least, she'd have some idea what her options were. How big would her subsidy be? What would her co-pays and deductibles be? But she has no idea, and the Obamacare snafus don't give her any confidence that she'll have a clearer answer by Dec. 15. Her biggest worry is going without coverage. So while Secretary of Health and Human Services Kathleen Sebelius says people have months to sign up for health care, that’s not true for Alice—she has seven weeks. No matter that Sebelius and her IT team assures everyone that the website will be fixed by Dec. 1. Alice has a reasonable belief that she’s going to get the wrong end of things. 

While healthcare.gov goes through its convalescence, Alice has tried to sort out things on her own. She is buffeted between government incompetence and insurance company greed. She can't get answers from healthcare.gov, and she's not sure she trusts the answers she hears from the insurance companies because she knows that they don't have her interests at heart. When they calculate her subsidy, is it the actual figure? Is it in their interest to steer her toward the best deal or the best deal for them? "I have to make this decision by Dec. 15, but I don't know if there will be real information by then," she says.

President Obama suggested that frustrated customers call the 800 number, so Alice did. The woman from healthcare.gov said she could mail her some information, which would arrive in three weeks. “Can't you email it to me?” Alice asked. We're not set up for that, the Obamacare operator replied. Once the application arrives, Alice is to fill out the forms, mail them back, and hope for speedy processing. It had better be fast because at that point there will only be about a month before her coverage lapses.

While Alice waits for the mail to arrive, she has tried healthcare.gov several more times to get any kind of additional information. According to the site, she has only two carriers to choose from in Pennsylvania and she can only learn the name of the plans and the estimated premium and nothing more—not the co-pays, deductibles, or anything else. She's on the comparison-shopping site but can't actually compare plans. "Not only are they quoting you a bogus number, but you can't see what that bogus number represents," she says. "You are in total bullshit land." Her insurer gives her a number for her new monthly premium, but it's several hundred dollars a month higher than the estimate she got on healthcare.gov.

Had Obamacare not existed, Alice would have renewed her expensive but passable plan and lived under the fear that her rates would increase. Now she has spent a month not sure what she's going to have and experiencing the dread that she’s going to get squashed in the pincer movement of the government and the private health insurance market. Whether you're in the private market or the employer system, the health care insurance dance is an unpleasant one. You study the forms, listen to the webinars, and try to read the fine print, but in the end you can never shake the feeling that you're losing out in some way you'll never understand until it's too late. In the end, Obamacare may be wonderful for Alice, but so far its rollout has only made her sick.

Source: http://www.slate.com/articles/news_and_politics/politics/2013/10/obamacare_worries_grow_will_private_insurance_end_before_the_government.html
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Ion Air Pro 3: The Waterproofiest Naked Action Cam Around

Ion may not be the first name you think of when it comes to action cameras (or the second), but the company has been around for a few years. The original Air Pro came in third in our action camera Battlemodo back in June of 2012, but it left us hungry for better image quality. The Air Pro 3 might just satisfy.

Read more...

Source: http://feeds.gawker.com/~r/gizmodo/full/~3/YegVcuOv09k/ion-air-pro-3-the-waterproofiest-naked-action-cam-arou-1454474921
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