Stargardt disease: the leading cause of juvenile macular degeneration

Stargardt disease is a rare yet life devastating condition. It is the most common form of inherited macular degeneration, and affects about one in 10,000 people (about 30,000 people in the United States).

There is currently no cure or treatment for Stargardt disease but clinical trials using gene and cell therapies are currently undergoing.

Patients with Stargardt most often start experiencing significant vision loss during their childhood and teenage years (60% of the patients are diagnosed before 20 years of age).

This vision loss cannot be corrected by glasses, and diagnosis had been traditionally delayed due to the young age of the patients and the rareness of the disease.

After diagnosis, and depending on age at onset, vision deteriorates progressively to levels below 20/200 (legal blindness).

The disease affects mostly the central vision and spares some of the peripheral vision, although there are very severe forms that lead to complete blindness.

Almost all of those affected by Stargardt disease will live legally blind during their adult lives, although patients with late onset may retain some visual acuity.

Loss of central vision leads to impossibility to perform tasks such as reading, writing, driving or recognizing faces.

In 1997, scientists discovered that Stargardt disease results from a defective gene, the ABCA4, responsible for the synthesis of an important protein called Rim protein.

A normally functioning Rim protein transports vitamin A molecules from the photoreceptors (the molecules sensitive to light) back into specialized cells (called RPE), where vitamin A molecules are recycled to be reused for vision.

In Stargardt, the defects in the ABCA4 gene lead to partial or full dysfunction of this protein. As a result, vitamin A transport is affected and vitamin A molecules tend to accumulate in the photoreceptors.

This accumulation leads to the formation of toxic pigments (known as "vitamin A dimers") believed to be partly responsible for vision loss.

Although normal individual also form vitamin A dimers, this process usually takes decades, explaining why age-related macular degeneration (AMD) occurs later in life of normal people, while the same process takes only a few years in Stargardt explaining vision loss from childhood.

Read more of this article here at Alkeus - Stargardt disease

Explore – The self is a compelling, seductive illusion

The self is something that is central to a lot of psychological questions and, in fact, a lot of psychologists have difficulty describing their work without positing the notion of a self. It’s such a common daily, profound, indivisible experience for most of us. Some people do manage to achieve states of divided self or anatta, no self, they’re really skilled Buddhists. But for the majority of us the self is a very compulsive experience. I happen to think it’s an illusion and certainly the neuroscience seems to support that contention.

British experimental psychologist Bruce Hood on essentialism, further confirming the idea that character is fluid and there is no such thing as a fixed self.

Flame: The Massive Spy Malware Infiltrating Iranian Computers

A massive, highly sophisticated piece of malware has been newly found infecting systems in Iran and elsewhere and is believed to be part of a well-coordinated, ongoing, state-run cyberespionage operation.

The malware, discovered by Russia-based anti-virus firm Kaspersky Lab, is an espionage toolkit that has been infecting targeted systems in Iran, Lebanon, Syria, Sudan, the Israeli Occupied Territories and other countries in the Middle East and North Africa for at least two years.

Dubbed “Flame” by Kaspersky, the malicious code dwarfs Stuxnet in size – the groundbreaking infrastructure-sabotaging malware that is believed to have wreaked havoc on Iran’s nuclear program in 2009 and 2010.

Although Flame has both a different purpose and composition than Stuxnet, and appears to have been written by different programmers, its complexity, the geographic scope of its infections and its behavior indicate strongly that a nation-state is behind Flame, rather than common cyber-criminals — marking it as yet another tool in the growing arsenal of cyberweaponry.

The researchers say that Flame may be part of a parallel project created by contractors who were hired by the same nation-state team that was behind Stuxnet and its sister malware, DuQu.

“Stuxnet and Duqu belonged to a single chain of attacks, which raised cyberwar-related concerns worldwide,” said Eugene Kaspersky, CEO and co-founder of Kaspersky Lab, in a statement.

“The Flame malware looks to be another phase in this war, and it’s important to understand that such cyber weapons can easily be used against any country.”

Early analysis of Flame by the Lab indicates that it’s designed primarily to spy on the users of infected computers and steal data from them, including documents, recorded conversations and keystrokes. It also opens a backdoor to infected systems to allow the attackers to tweak the toolkit and add new functionality.

The malware, which is 20 megabytes when all of its modules are installed, contains multiple libraries, SQLite3 databases, various levels of encryption — some strong, some weak — and 20 plug-ins that can be swapped in and out to provide various functionality for the attackers.

It even contains some code that is written in the LUA programming language — an uncommon choice for malware.

Kaspersky Lab is calling it “one of the most complex threats ever discovered.”

“It’s pretty fantastic and incredible in complexity,” said Alexander Gostev, chief security expert at Kaspersky Lab.

Flame appears to have been operating in the wild as early as March 2010, though it remained undetected by antivirus companies.

“It’s a very big chunk of code. Because of that, it’s quite interesting that it stayed undetected for at least two years,” Gostev said. He noted that there are clues that the malware may actually date back to as early as 2007, around the same time-period when Stuxnet and DuQu are believed to have been created.

Gostev says that because of its size and complexity, complete analysis of the code may take years.

“It took us half-a-year to analyze Stuxnet,” he said. “This is 20-times more complicated. It will take us 10 years to fully understand everything.”

Kaspersky discovered the malware about two weeks ago after the United Nations’ International Telecommunications Union asked the Lab to look into reports in April that computers belonging to the Iranian Oil Ministry and the Iranian National Oil Company had been hit with malware that was stealing and deleting information from the systems.

The malware was named alternatively in news articles as “Wiper” and “Viper,” a discrepancy that may be due to a translation mixup.

Kaspersky researchers searched through their reporting archive, which contains suspicious filenames sent automatically from customer machines so the names can be checked against whitelists of known malware, and found an MD5 hash and filename that appeared to have been deployed only on machines in Iran and other Middle East countries.

As the researchers dug further, they found other components infecting machines in the region, which they pieced together as parts of Flame.

Kaspersky, however, is currently treating Flame as if it is not connected to Viper, and believes it is a separate infection entirely. The researchers dubbed the toolkit “Flame” after the name of a module inside it.

Read more here

RSA Animate - The Power of Networks - YouTube

In this new RSA Animate, Manuel Lima, senior UX design lead at Microsoft Bing, explores the power of network visualisation to help navigate our complex modern world.

Taken from a lecture given by Manuel Lima as part of the RSA's free public events programme. Listen to the full talk: The-power-of-networks...

Alain de Botton: A kinder, gentler philosophy of success - YouTube

Alain de Botton examines our ideas of success and failure -- and questions the assumptions underlying these two judgments. Is success always earned? Is failure? He makes an eloquent, witty case to move beyond snobbery to find true pleasure in our work.

End Malaria - YouTube

Buy the book at www.EndMalariaDay.com and save a life.

It isn't very often that a book has the power to save a life. Yes, good books can improve lives, shape lives, even change lives. But when was the last time a book literally helped save a life? If you're reading this page, the answer is right now.

This year, The Domino Project set out to change the future of publishing, and now it’s out to change the future of philanthropy.

The project’s latest release, by author Michael Bungay Stanier of Box of Crayons fame, is out to tackle one of our civilization’s grimmest epidemics: malaria. (And if the gravity of the issue still hasn’t stopped you dead in your tracks — like, for instance, the fact that a child dies of malaria every 45 seconds — watch Bill Gates’ 2009 TED talk.)

End Malaria: Bold Innovation, Limitless Generosity, and the Opportunity to Save a Life, released on End Malaria Day today, is a fantastic anthology that will save lives — by helping you be better, smarter, more efficient at your job.

The book features essays, tips and insights on great work by 62 leading writers and thinkers — including everyones' favourites Sir Ken Robinson, Brené Brown, Kevin Kelly, Scott Belsky, Barry Schwartz, Daniel Pink, Derek Sivers and more — with $20 out of every $25 book sale (that’s 80%, for the mathematically challenged) going to Malaria No More to buy mosquito nets for Africa, still the most effective malaria prevention method. (For comparison purposes, most product-based charitable contributions are in the 5-10% range.)

Milton Glaser: The Fear of Failure

A characteristic of artistic education is for people to tell you that you’re a genius. [...] So everybody gets this idea, if you go to art school, that you’re really a genius. Sadly, it isn’t true. Genius occurs very rarely. So the real embarrassing issue about failure is your own acknowledgement that you’re not a genius, that you’re not as good as you thought you were. [...] There’s only one solution: You must embrace failure. You must admit what is. You must find out what you’re capable of doing, and what you’re not capable of doing. That is the only way to deal with the issue of success and failure because otherwise you simply would never subject yourself to the possibility that you’re not as good as you want to be, hope to be, or as others think you are.” ~ Milton Glaser

People Who Let Go Unachievable Goals Have Better Quality of Life

Reaching for the Moon? People who let go of unachievable old goals have a better quality of life, McGill University researchers have discovered. The discovery was made when they were studying data of breast cancer patients.

Researchers had studied 176 breast cancer survivors between the ages of 28 and 79. Self-reports of the individual's capacity to adjust their goals were measured at the start of the study. At the same time, researchers measured self-report of physical activity, sedentary activity, emotional well-being, and daily physical symptoms such as nausea and pain.

Three months later, they took a look at another round of self-reports. The study found that goal reengagement (being able to set new goals) was associated with more physical activity, increased emotional well-being and fewer physical symptoms. In addition, breast cancer survivors who were able to let go of old goals and to find new ones were less sedentary, which contributed to improved well-being. These findings support earlier research showing that goal adjustment can influence better well-being and health.

"By engaging in new goals a person can reduce the distress that arises from the desire to attain the unattainable, while continuing to derive a sense of purpose in life by finding other pursuits of value," said Carsten Wrosch, researchers at Concordia University's Department of Psychology and Centre for Research in Human Development. "Abandoning old goals allows someone to invest sufficient time and energy in effectively addressing their new realities."

Researchers have also suggested that breast cancer survivors should engage in at least 150 minutes of moderate to vigorous-intensity physical activity every week to gain health benefits.

"It is safe, feasible and effective for enhancing well-being and health among breast cancer survivors," said Catherine Sabiston, researcher at the McGill University. "Unfortunately, few survivors are engaging in the recommended levels of activity."

"Our research reveals that the capacity to adjust goals plays a pivotal role in facilitating not only high physical activity but also low sedentary activity and thereby contributing to overall improved well-being," said Wrosch. "Given that it is possible to influence adjustment to specific goals; it may be beneficial to integrate goal adjustment processes into clinical practice."

How to design the best business card [infographic]

How to design the best business card [infographic]