Liberty Media CEO Doubts HBO Will See Much Over-the-Top Success

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Updated from March 5 to include potential partnerships in the sixth paragraph.

NEW YORK (TheStreet) -- Liberty Media (LMCA - Get Report)CEO Greg Maffeiturned up the heat this week in the long-simmering competition between cable-TV's premium channels.

In a shot at rival Time Warner (TWX - Get Report), Maffei told investors at a conference hosted by Morgan Stanley that HBO shouldn't expect to generate much demand or profit from its Internet-based standalone service, which is expected to launch later this year.

Conversely,Starz (STRZA), of which Maffei is chairman through Liberty's inter-locking corporate structure, is better positionedthan HBO to see profit from alternative offerings from traditional cable-TV and satellite distributors, he said.

"It's unclear to me that there's a massive amount of over-the-top demand for HBO," Maffei said, expressing doubt that consumers will be willing to pay anywhere around $15 for a non-bundled premium-channel service. "It's not clear to me that that's going to create enormous amount of incremental demand for HBO. What is clear to me is that there are people who don't want to get behind the bundle."

In October, HBO CEO Richard Plepler announced plansto sell online subscriptions tothe channel as an Internet-based offering. The goal, Plepler said, was to win over the10 million U.S. homes that have an Internet connection but don't subscribe to pay-TV as well as the 70 million homes that get pay-TV but don't get HBO.

But HBO's strategy could run into resistance from cable-TV providers who fear that the standalone offering could cannibalize their overall service, saidPivotal Research Group analyst Jeffrey Wlodarczak.

"HBO's hope is that by not being part of the more expensive digital package they will be better able to expand their market," Wlodarczaksaid in an e-mail. "As HBO tries to go direct to the consumer there is no ability for HBO to capture extra margin because today distributors make no money on HBO."

Yet HBO carries more leverage with cable-TV and satellite operators than its rivals at Starz or CBS's (CBS) Showtime, saysShahid Khan, co-founderofMediamorph, the New York-based media industry software and data provider. Time Warner will be faced with additional technology and billings related to brining HBO online, but subscriber growth will largely turn into profits. "There are additional costs to unbundling but you can pretty much use the infrastructure that you use for your TV Everywhere structure -- it's not that much more," Khan said in a phone interview. "HBO isthe highest leveraged premium channel out there, so they can afford to piss off the [cabel-TV] partners. The others, like Starz, may not be able to." Cable-TV distributors, Wlodarczak added, profit handsomely from Starz, and with HBO going direct through an online offering, distributors will be incentivized to push alternative packages that include premium channels. Under CEO Chris Albrecht, Starz has increased its production of original programming, including serials such asOutlander and Black Sails, following the transition amongpremium cable-TV channels from libraries of films and TV serials into networks of production and acquisitions. Premium networks such as HBO and Starz, Maffei said, should be most concerned with "cord cutters" who are dropping their cable or satellite services completely, and "cord shavers," who are reducing their bundled services. Such consumers may consider the HBO over-the-top offering, but find that they would prefer to pay for Netflix (NFLX), which offers a wide variety TV serials, films and increasingly, its own content. An HBO spokesman declined to comment. Starz, Maffei said, may choose not to offer an online service but instead roll out a different product in conjunction with its cable, telco and satellite partners. Maffei didn't elaborate on what that project might be. That's a strategyWlodarczak said could be successful.

If Starz' original content effort is highly successful "they have the opportunity longer term to go direct and capture a lot of margin going to distributors," he said. "Realistically, the best move for Starz is to be the best partner possible to their distributors." Must Read: Warren Buffett's Top 10 Stock Buys

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Liberty Media CEO Doubts HBO Will See Much Over-the-Top Success

Liberty Media CEO Takes Aim at HBO, Skeptical of Internet Service

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Liberty Media CEO Greg Maffei is taking aim at longtime competitor Time Warner, saying he doesn't expect its premium channel HBO to generate much demand or profit from its Internet-based standalone service expected later this year. Maffei, chairman of rival Starz, told investors at a conference this week that he is doubtful consumers will be willing to pay anywhere around $15 for the non-bundled service, according to TheStreet's Jeff Berman. Consumers who are trying to save money by dropping channels may consider the HBO over-the-top offering, Maffei said, but find they would prefer to pay for Netflix instead, which plans to boost its original programming next year. Also, he said there doesn't seem to be much room for incremental profit from the service.

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Liberty Media CEO Takes Aim at HBO, Skeptical of Internet Service

What makes Rand Paul strange

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Put into practice, libertarianism can make a mess. If parents have the right to endanger others by not getting their children immunized, why cant individuals decide whether theyre too drunk to drive? writes syndicated columnist Froma Harrop.

U.S. Sen. Rand Paul believes that vaccinating children should be up to the parents, an increasingly unpopular view after recent outbreaks of measles, mumps and other diseases. And throwing a newts eye of quack science into the vat, the Kentucky Republican promotes the myth that these shots put children at risk.

The political results have been toil and trouble.

Its not easy being a politician and a principled libertarian. One who believes in the primacy of individual freedom often takes stances far from the mainstream. It is the true libertarians lot to be unconventional, to bravely accept unwanted consequences in the name of liberty. By not going that extra philosophical mile and adding junk science to the mix Paul comes off as merely weird.

He was already fighting blowback when he ventured into an interview with CNBCs Kelly Evans.

Well, I guess being for freedom would be really unusual, he responded to a question about whether vaccinations should be voluntary. I dont understand why that would be controversial.

Does he not? Then he again gave credence to crazy talk of healthy children ending up with profound mental disorders after being vaccinated.

When the chat moved to taxes and Evans challenged some of his statements, he shushed her as if she were a little girl. Calm down a bit here, Kelly, he said.

Clearly, it wasnt Kelly who needed calming.

By the end, Paul had accused Evans of being argumentative and blamed the media for distorting positions he had left purposely vague. Not his finest hour.

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What makes Rand Paul strange

Thats something that should make libertarians nervous: Inside the tumultuous rise of an American ideology

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Libertarianism, like its ideological cousin neoliberalism, is one of those words that people in the political world use a lot without establishing whether everyone agrees on its meaning. This doesnt really matter in the vast majority of cases (because nothing that happens during a fight in a comment thread or on Twitter matters). But as support for libertarian-backed causes like marriage equality, opposition to the war on drugs, and resistance against the rise of mass incarceration become ever-greater parts of U.S. politics, the definition of libertarianism will matter more, too for the sake of apportioning credit and blame, if nothing else.

In the interest of nailing down a famously elusive and controversial term, then, Salon recently spoke over the phone with David Boaz, longtime member of the influential and Koch-founded Cato Institute think tank and author of Libertarianism: A Primer, which was just updated and rereleased as The Libertarian Mind: A Manifesto for Freedom. Our discussion touched on the big issues mentioned above, as well as Boazs thoughts on what liberals and conservatives misunderstand about libertarianism, and why he thinks his favored political philosophys future is so bright. Our conversation is below and has been edited for clarity and length.

If you had to pick one defining or differentiating characteristic of the libertarian mind, what would it be?

The first line of the book says that libertarianism is the philosophy of freedom, so what distinguishes libertarians is their commitment to freedom. That can manifest itself in lots of different issues, from marijuana and gay marriage to smaller government and lower taxes, but the fundamental idea of freedom as the proper political condition for society is the thing that unites libertarians.

Wouldnt most Americans say they care deeply about freedom, though? So is it the definition of freedom that distinguishes libertarianism from liberalism and conservatism? Or is it where freedom ends up in the hierarchy of values?

In America, virtually everybody comes out of the classical liberal tradition. The classical liberal tradition of John Locke, Adam Smith, Thomas Jefferson, and John Stuart Mill stresses freedom under law and limited government and most Americans share that. The difference with libertarians is that we do make freedom our political priority. Freedom is not necessarily any persons primary value. Your primary value may be courage or friendship or love or compassion or the arts; but freedom is the primary political value for libertarians.

I do think that is a difference between libertarians and liberals or conservatives who value freedom but also value other things. Modern American liberals would say, I believe, that they value equality along with freedom. Libertarians would tend to respond, well, in the real world you get more equality when you have freedom and free markets, though libertarians certainly believe in equal rights and equal freedom. Some conservatives value doing Gods will or maintaining social order or maintaining tradition along with freedom.

In that sense, I do think libertarians put freedom at the center of their political philosophy in a way that many liberals and conservatives do not.

If you had to pick one thing about libertarianism that liberals misunderstand the most, what would it be?

I think there is first a misunderstanding that libertarians are conservatives and I think thats wrong. Libertarians are classical liberals. We trace our heritage back to, not the aristocracy or established church, but to the liberal thinkers and activists who challenged those institutions.

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Thats something that should make libertarians nervous: Inside the tumultuous rise of an American ideology

Libertarianism is for petulant children: Ayn Rand, Rand Paul and the movements sad rebellion

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This article originally appeared on AlterNet.

Libertarians believe themselves controversial and cool. Theyre desperate to package themselves as dangerous rebels, but in reality they are champions of conformity. Their irreverence and their opposition to political correctness is little more than a fashion accessory, disguising their subservience tofor all their protests against the political elitethe real elite.

Ayn Rand is the rebel queen of their icy kingdom, villifying empathy and solidarity. Christopher Hitchens, in typical blunt force fashion, undressed Rand and her libertarian followers, exposing their obsequiousness toward the operational standards of a selfish society: I have always found it quaint, and rather touching, that there is a movement in the US that thinks Americans are not yet selfish enough.

Libertarians believe they are real rebels, because theyve politicized the protest of children who scream through tears, Youre not the boss of me. The rejection of all rules and regulations, and the belief that everyone should have the ability to do whatever they want, is not rebellion or dissent. It is infantile navet.

As much as libertarians boast of having a political movement gaining in popularity, youre not the boss of me does not even rise to the most elementary level of politics. Aristotle translated politics into meaning the things concerning the polis, referring to the city, or in other words, the community. Confucius connected politics with ethics, and his ethics are attached to communal service with a moral system based on empathy. A political program, like that from the right, that eliminates empathy, and denies the collective, is anti-political.

Opposition to any conception of the public interest and common good, and the consistent rejection of any opportunity to organize communities in the interest of solidarity, is not only a vicious form of anti-politics, it is affirmation of Americas most dominant and harmful dogmas.In America, selfishness, like blue jeans or a black dress, never goes out of style. It is the style. The founding fathers, for all the hagiographic praise and worship they receive as ritual in America, had no significant interest in freedom beyond their own social station, regardless of the poetry they put on paper. Native Americans, women, black Americans, and anyone who did not own property could not vote, but taxation without representation was the rallying cry of the revolution. The founders reacted with righteous rage to an injustice to their class, but demonstrated no passion or prioritization of expanding their victory for liberty to anyone who did not look, think, or spend money like them.

Many years after the nations establishment as an independent republic, President Calvin Coolidge quipped, The chief business of the American people is business. It is easy to extrapolate from that unintentional indictment how, in a rejection of alternative conceptions of philosophy and morality, America continually reinforced Alexis De Tocquevilles prescient 1831 observation, As one digs deeper into the national character of Americans, one sees that they have sought the value of everything in this world only in the answer to this single question: How much money will it bring in?

The disasters of reducing life, the governance of affairs, and the distribution of resources to such a shallow standard leaves wreckage where among the debris one can find human bodies. Studies indicate that nearly 18,000 Americans die every year because they lack comprehensive health insurance. Designing a healthcare system with the question, How much money will it bring in? at the center, kills instead of cures.

The denial of the collective interest and communal bond, as much as libertarians like to pose as trailblazers, is not the road less traveled, but the highway in gridlock. Competitive individualism, and the perversion of personal responsibility to mean social irresponsibility, is what allows for America to limp behind the rest of the developed world in providing for the poor and creating social services for the general population.

It also leads to the elevation of crude utility as a measurement of anythings purpose or value. Richard Hofstadter, observed in his classicAnti-Intellectualism in American Life, that many Americans are highly intelligent, but their intelligence is functional, not intellectual. They excel at their occupational tasks, but do not invest the intellect or imagination in abstract, critical, or philosophical inquiries and ideas. If society is reducible to the individual, and the individual is reducible to consumer capacity, the duties of democracy and the pleasures of creativity stand little chance of competing with the call of the cash register.

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Libertarianism is for petulant children: Ayn Rand, Rand Paul and the movements sad rebellion

21 Rand Paul quotes that expose libertarianism for the con job it is

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This article originally appeared on AlterNet.

Senator Rand Paul, of Kentucky, seems to have no problem contradicting himself. The self-proclaimed constitutional conservative is typically lost in libertarian thought leading him to make inflammatory sexist, racist and overbearingly hypocritical comments on nearly every issue he faces. Whether hes attempting to police womens bodies, ignoring police brutality for stingy tobacco taxes, or speaking out against vaccines and posting himself receiving booster shots only days later, Ron Pauls son is one politician you can unabashedly hate or enjoy laughing at.

1. When Paul spoke outagainst vaccines:

I have heard of many tragic cases of walking, talking normal children who wound up with profound mental disorders after vaccines.

Click to enlarge.

Rand Paul /Twitter

2. When he backedvoter ID laws:

I dont think theres a problem with showing your ID, but I do think theres a problem with Republicans saying, Hey, our big issue for the campaign is going to be voter ID, because what it creates is a lot of African-Americans understandably remember the 40s and 50s in the South, and they remember suppression of the vote.

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21 Rand Paul quotes that expose libertarianism for the con job it is

My libertarian vacation nightmare: How Ayn Rand, Ron Paul & their groupies were all debunked

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Last month, I spent my final vacation night in Honduras in San Pedro Sula, considered the most dangerous city outside of the war-torn Middle East. I would not have been scared, except that I traveled with my wife and our four children, aged 5, 7, 14 and 18. On our last taxi ride, we could not find a van to fit us all, so we rode in two taxis. Mine carried me and my two daughters, aged 5 and 14, while the driver blasted Willie Nelson singing City of New Orleans (a city that is also considered very dangerous).

It was a surreal moment, traveling in one of the most dangerous cities in the world with my babies in tow. I gave a nod to the radio. Willie, I said, and he gave me a grin and vigorous s. Theres a lot of American cowboy culture in Honduras, but along with silly hats, Honduras has also taken one of our other worst ideaslibertarian politics. By the time Id made it to San Pedro Sula, Id seen much of the countryside and culture. Its a wonderful place, filled with music, great coffee, fabulous cigars and generous people, but its also a libertarian experiment coming apart.

People better than I have analyzed the specific political moves that have created this modern day libertarian dystopia. Mike LaSusa recently wrote a detailed analysis of such,laying out how the bad ideas of libertarian politics have been pursued as government policy.

In America, libertarian ideas are attractive to mostly young, white men with high ideals and no life experience that live off of the previous generations investments and sacrifice. I know this because as a young, white idiot, I subscribed to this system of discredited ideas: Selfishness is good, government is bad. Take what you want, when you want and however you can. Poor people deserve what they get, and the smartest, hardworking people always win. So get yours before someone else does. I read the books by Charles Murray and have an autographed copy of Ron Pauls The Revolution. The thread that links all the disparate books and ideas is that they fail in practice. Eliminate all taxes, privatize everything, load a country up with guns and oppose all public expenditures, you end up with Honduras.

In Honduras, the police ride around in pickup trucks with machine guns, but they arent there to protect most people. They are scary to locals and travelers alike. For individual protection theres an army of private, armed security guards who are found in front of not only banks, but also restaurants, ATM machines, grocery stores and at any building that holds anything of value whatsoever. Some guards have uniforms and long guns but just as many are dressed in street clothes with cheap pistols thrust into waistbands. The country has a handful of really rich people, a small group of middle-class, some security guards who seem to be getting by and a massive group of people who are starving to death and living in slums. You can see the evidence of previous decades of infrastructure investment in roads and bridges, but its all in slow-motion decay.

I took a van trip across the country, starting in Copan (where there are must-see Mayan ruins), across to the Caribbean Sea to a ferry that took my family to Roatan Island. The trip from Copan to the coast took a full six hours, and we had two flat tires. The word treacherous is inadequatea better description is post-apocalyptic. We did not see one speed limit sign in hundreds of kilometers. Not one. People drive around each other on the right and left and in every manner possible. The road was clogged with horses, scooters and bicycles. People traveled in every conceivable manner along the crumbling arterial. Few cars have license plates, and one taxi driver told me that the private company responsible for making them went bankrupt. Instead of traffic stops, there are military check points every so often. The roads seemed more dangerous to me than the gang violence.

The greatest examples of libertarianism in action are the hundreds of men, women and children standing alongside the roads all over Honduras. The government wont fix the roads, so these desperate entrepreneurs fill in potholes with shovels of dirt or debris. They then stand next to the filled-in pothole soliciting tips from grateful motorists. That is the wet dream of libertarian private sector innovation.

On the mainland there are two kinds of neighborhoods, slums that seem to go on forever and middle-class neighborhoods where every house is its own citadel. In San Pedro Sula, most houses are surrounded by high stone walls topped with either concertina wire or electric fence at the top. As I strolled past these castle-like fortifications, all I could think about was how great this city would be during a zombie apocalypse.

On a previous vacation abroad, Id met a resident of San Pedro Sula by the name of Alberto. Through Facebook, we connected up to have drinks and share a short tour of his home city. A member of the small, dwindling middle class, Alberto objects to his city being labeled the most dangerous in the Western Hemisphere. He showed me a few places in the city that could have been almost anywhere, a hipster bar, a great seafood place (all guarded by armed men, of course). Alberto took me on a small hike to a spot overlooking the city and pointed out new construction and nice buildings. There are new buildings and construction but it is funded exclusively by private industry. He pointed out a place for a new airport that could be the biggest in Central America, he said, if only it could get built, but there is no private sector upside. Alberto made me see the potential, the hope and even the hidden beauty of the place.

For our last meal in San Pedro Sula, my family walked a couple blocks from our fortress-like bed and breakfast to a pizza restaurant. It was the middle of the day and we were the only customers. We walked through the gated walls and past a man in casual slacks with a pistol belt slung haphazardly around his waist. Welcome to an Ayn Rands libertarian paradise, where your extra-large pepperoni pizza must also have an armed guard.

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My libertarian vacation nightmare: How Ayn Rand, Ron Paul & their groupies were all debunked

Are You a Libertarian? Take This Quiz

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Somewhere between 30 and 40 million Americans hold libertarian views. Are you one of them?Takethis quiz excerpted fromDavid Boazs new bookThe Libertarian Mind: A Manifesto for Freedomand find out just how libertarian you really are!

The quiz launched just over a week ago and almost 30,000 people have already taken it. Have you?Use #LibertarianMind to share yourresults on social media, tag your friends, and see how they measure up!

Of course, the quiz represents a very simplified version of libertarian principles andvery few people will have perfect scores in any one direction, but its a great way to open up a discussion with friends and family. If that discussion leads to more curiosityabout libertarianism and its principles, point them in our directionor give them a their very own copy ofThe Libertarian Mind!

Havent had a chance to readThe Libertarian Mindyet?We have a limited number of copiesto give away.Take the quizto learn the details! You can also follow The Libertarian Mind on Facebook for news on the book, media appearances, and more.

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Are You a Libertarian? Take This Quiz

Carnival Legend Cruise Ship Tour, 2015 Australia to South Pacific Islands. – Video

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Carnival Legend Cruise Ship Tour, 2015 Australia to South Pacific Islands.
Carnival Legend Ship Tour 2015. From aft to forward different levels and whats available on board. Cruise was from Australia to the south pacific, 11 nights. This is a slideshow as I had big...

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Carnival Legend Cruise Ship Tour, 2015 Australia to South Pacific Islands. - Video

Apostle Islands ice caves in Wisconsin could be open this weekend

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Apostle Islands National Lakeshore officials say the popular ice caves could be open to the public this weekend.

Lakeshore planning chief Julie Van Stappen says they'll check the ice Wednesday, then make the call. The ice must be locked in and thick enough on the trail out to the mainland ice caves.

High winds are in the forecast for the area this week. TheSt. Paul Pioneer Press reports Van Stappen says it's hoped the extensive ice cover on the lake will protect the ice cave formations from breaking up.

The caves were open to the public last winter for the first time since 2009. An estimated 138,000 people visited the caves last season.

Associated Press

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Apostle Islands ice caves in Wisconsin could be open this weekend

Beginner's guide to the Canary Islands: The other side of Spain

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Mount Teide on Tenerife soars 3719 metres above sea level.Photo: 123rf.com

Flicking through El Mundo a top Spanish newspaper one February morning, I'm struck by images of Madrid blanketed in snow and Madrilenos shivering in thick coats and scarves.

I'm in another part of Spain, in a T-shirt, shorts and thongs, sipping a cafe con leche on a cobbled plaza framed by handsome balconied mansions dripping with radiant bougainvillea. Pretty soon, the sun will melt into the Atlantic Ocean, happy hour will commence, and the smell of barbecued seafood and the sound of tapas orders, wine-fuelled chatter and jazz, salsa and flamenco music will spike the blissfully mild air.

Such is life on the Canary Islands. Moored off southern Morocco, this volcanic archipelago was initially the preserve of the wealthy and wanderlustful who would sail here for the sub-tropical rays and therapeutic waters. The Canaries went mass-market in the 1970s and 80s, however, with resorts, Anglo-Saxon pubs and Nordic restaurants mushrooming along coastlines, sparking an influx of holidaymakers seeking sun, sea, golf and R&R away from the frosty climes of Manchester, Berlin and Copenhagen. Despite their appeal to Europeans particularly during winter, when temperatures are usually in the early to mid 20Cs the Canaries remain a mystery to Australians, who are rarely tempted to take the three-hour flight here from Madrid. Trust me it's definitely worth the effort. Get beyond the islands' (mostly) uninspiring tourist enclaves, and countless cultural treats and pulse-raising surprises await the adventurous traveller.

The largest of the seven main Canaries, Tenerife is 46 times smaller than Tasmania and distinctly two-faced. The south is parched, Mars-like and smothered in cacti-strewn desert; the lusher, more humid north is carpeted in banana plantations, pine forests and vineyards. In the middle of the island looms the cause of these micro-climates.

Spain's loftiest peak, and the third highest volcano in the world (when measuring its base from the ocean), Mount Teide soars 3719m above sea level and is dusted with snow in winter. The easiest way up Teide is via a cable car that ascends to a viewpoint, from which, depending on the weather, you'll either observe a sea of clouds, or the entire Canarian archipelago. Scaling the mountain on foot takes five hours and decent hiking boots, sunblock and plenty of water but it's infinitely more rewarding.

Apart from July and August, when temperatures can hit the mid-30Cs, Tenerife is a magnificent walking destination. Sign-posted trails, for all fitness levels, zig-zag the island. I love the Masca gorge hike, which snakes 8.5km from the idyllic whitewashed village of Masca down to its rocky beach, where you can board a boat to the town of Los Gigantes.

The next day I laze on Tenerife's nicest beach. A contrast to the island's many volcanic black-sand coves, Playa Las Teresitas flaunts golden sands imported from the Sahara desert. It's near Santa Cruz, Tenerife's chilled-out capital and port, which springs to life each February when it hosts what's claimed to be the planet's biggest carnival after Rio de Janeiro's.

A 30-minute tram ride from Santa Cruz whose avant-garde auditorium was designed by ace Spanish architect Santiago Calatrava La Laguna is an old charmer.

Its cobblestone grid-centre, crammed with museums, galleries, churches, colleges, convents and palaces; has UNESCO World Heritage listed status and was the blueprint for the Spanish colonial towns of Latin America. Indeed, on a foggy, drizzly day, there are shades of Quito, the Ecuadorian Andean capital, about La Laguna, which was established, 543 metres above sea level, in AD1494, after a Spanish army conquered the Guanches (the native people of the Canary islands).

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Beginner's guide to the Canary Islands: The other side of Spain

New genetic syndrome found, tied to errors in 'master switch' during early development

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IMAGE:Dr. Ian D. Krantz is the co-director of the Individualized Medical Genetics Center at The Children's Hospital of Philadelphia. view more

Credit: The Children's Hospital of Philadelphia

Analyzing a puzzling multisystem disorder in three children, genetic experts have identified a new syndrome, shedding light on key biological processes during human development. The research also provides important information to help caregivers manage the disorder, and may offer clues to eventually treating it.

"This syndrome illuminates a very important pathway in early human development--a sort of master switch that controls many other genes," said study leader Ian D. Krantz, M.D., co-director of the Individualized Medical Genetics Center at The Children's Hospital of Philadelphia (CHOP). Krantz, a medical geneticist, is an attending physician in CHOP's comprehensive human genetics program.

Krantz is the senior author of the study, published online today in Nature Genetics. His co-study leader is Katsuhiko Shirahige, Ph.D., of the Institute for Molecular and Cellular Biosciences, University of Tokyo, also the home institution of first author Kosuke Izumi.

The investigators named the disorder CHOPS syndrome, with the acronym representing a group of symptoms seen in the affected children: cognitive impairment and coarse facies (facial features), heart defects, obesity, pulmonary involvement, short stature and skeletal dysplasia (abnormal bone development).

The central research finding is that mutations in the gene AFF4 disrupt a crucial group of proteins called the super elongation complex (SEC). The SEC controls the transcription process by which DNA is copied into RNA, enabling genes to be expressed in a developing embryo. The timing of this biological process is tightly regulated, so anything that interferes with this timing can disturb normal development in a variety of ways.

"Because the SEC involves such a crucial process in cell biology, it has long been a focus of study, particularly in cancer," said Krantz. "CHOPS syndrome is the first example of a human developmental disorder caused by germline mutations in the SEC."

Originating in the embryo, germline mutations are passed along to every cell in a developing organism, with harmful effects in multiple organs and biological systems. The mutated AFF4 gene produces mutated proteins, which then accumulate and cause a cascade of abnormalities in other genes controlled by AFF4.

"AFF4 has a critical role in human development, regulating so many other genes," said Krantz. "When it is mutated, it can damage the heart and skeleton, and lead to intellectual disability, among other effects."

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New genetic syndrome found, tied to errors in 'master switch' during early development

Dr Alvarez Shares What Led Him To Bariatric Surgery | Gastric Sleeve Doctor | Endobariatric – Video

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Dr Alvarez Shares What Led Him To Bariatric Surgery | Gastric Sleeve Doctor | Endobariatric
http://www.endobariatric.com #GastricSleeveDoctor Dr. Alvarez introduces himself and gives a brief description of his practice and why weight loss surgery. Top notch health...

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Dr Alvarez Shares What Led Him To Bariatric Surgery | Gastric Sleeve Doctor | Endobariatric - Video