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Researchers Find How Mutant Gene Can Cause Deafness

Scientists at The Scripps Research Institute (TSRI) have discovered how one gene is essential to hearing, uncovering a cause of deafness and suggesting new avenues for therapies.

The new study, published November 20 in the journal Neuron, shows how mutations in a gene called Tmie can cause deafness from birth. Underlining the critical nature of their findings, researchers were able to reintroduce the gene in mice and restore the process underpinning hearing.

“This raises hopes that we could, in principle, use gene-therapy approaches to restore function in hair cells and thus develop new treatment options for hearing loss,” said Professor Ulrich Müller, senior author of the new study, chair of the Department of Molecular and Cellular Neuroscience and director of the Dorris Neuroscience Center at TSRI.

The Gene Responsible

The ear is a complex machine that converts mechanical sound waves into electric signals for the brain to process. When a sound wave enters the ear, the uneven ends (stereocilia) of the inner ear’s hair cells are pushed back, like blades of grass bent by a heavy wind. The movement causes tension in the strings of proteins (tip links) connecting the stereocilia, which sends a signal to the brain through ion channels that run through the tips of the hair cell bundles.

This process of converting mechanical force into electrical activity, called mechanotransduction, still poses many mysteries. In this case, researchers were in the dark about how signals were passed along the tip links to the ion channels, which shape electrical signals.

To track down this unknown component, researchers in the new study built a library of thousands of genes with the potential to affect mechanotransduction.

The team spent six months screening the genes to see if the proteins the genes produced interacted with tip link proteins. Eventually, the team found a gene, Tmie, whose protein, TMIE, interacts with tip link proteins and connects the tip links to a piece of machinery near the ion channel.

A Path to New Treatments

This discovery answers a long-standing question in neuroscience. Scientists have long known that mutations in the Tmie gene could cause deafness—but they weren’t sure how.

Once they found that TMIE plays a role bridging the tip links and ion channels, the researchers bred a population of “knock-out mice” that lacked the gene. They examined the hair cells of the mice with electrophysiological techniques and found that without TMIE, no electrical signal could be evoked in hair cells after stimulation.

“The mechanotransduction current is gone; the mouse is totally deaf,” explained Bo Zhao, a research associate in the Müller lab and first author of the new paper.

In a second experiment, the researchers reintroduced Tmie to mice that had been deaf since birth and found the electrical signals were restored.

Müller said the next challenge is to find out how the individual components of the complex mechanotransduction system form in hair cells. “We would also like to understand the biophysical principles by which these proteins convert mechanical signals into electrical signals,” he said.

MAY 2014

The games market held relatively steady this month in terms of volume, with another 14 titles tracked in Nielsen’s VGT for retail release in May. Perhaps the most notable release this month, in terms of consumer expectations, is Ubisoft’s Watch Dogs. The open world action-thriller has built up a considerable amount of anticipation from both years of marketing as well as a number of delays to its release date. Another higher profile release this month is Bethesda’s Wolfenstein: The New Order. Continuing to build on the Castle Wolfenstein franchise that originated in the early 1980s, this first-person shooter is set in alternative history 1960s Europe in which the Nazis won the Second World War.

Nintendo’s garden had a fair number of May blossoms for Nintendo fans this month. Early in the month, 3DS gamers were presented with another Mario sports title in Mario Golf: World Tour, as well as an extension of the beloved Kirby franchise in Kirby: Triple Deluxe. And then the month ended with the first Wii U title from the genre-defining kart racing series; Mario Kart 8.

Gamers on the Sony platforms also had reason for Spring cheer this month as well, as they saw two incredibly popular titles ported over to their devices. Borderlands 2 expanded the shoot-n-loot world of Pandora onto the PS Vita for the first time in May. And Minecraft, which had already expanded its sandbox world to the PS3 via the PlayStation Network with Minecraft: PlayStation Edition, doubled down on the platform with a retail disc release this month.


Co-Authored By Carl Kulo & Leigh Wright

Through May 18, 2014, sales tracked through the Nielsen BookScan US measurement service were strong – erasing the 3.7 overall year-over-year decline reported through Q1 2014.

Traditional book retailer business grew, while the Mass Merchandiser distribution channel went from 12% down to 3% down since the end of March.

Adult Fiction continues its downward trend (-13% across the board) even though small gains are being reported from the Graphic Novel and Western categories. Adult Non-Fiction is flat, while Juvenile book sales continue to set the pace for the industry – up 13% YTD.

Below is a list of the overall Nielsen top sellers through Week 20, 2013:



Author Spotlight

Fittingly, this month’s spotlight is on John Green and Veronica Roth, who have been dominating the Nielsen Juvenile Fiction bestseller charts (tying up five of the top ten overall bestseller slots). This should be no surprise, seeing as they’ve been dominating Hollywood this spring as well. Roth’s Divergent made a strong showing at the box office after its March 21st opening, and her trilogy’s sales reflect that, with Insurgent, Divergent, and Allegiant holding steady at spots 4, 5, and 6 respectively for week 25; all told, this series has sold over 2.6 million copies this year. Sales of Green’s The Fault in Our Stars reflects similar enthusiasm for the upcoming film adaptation, which opens nationwide on June 6th. The original paperback and movie tie-in paperback have been sitting at positions 1 and 2 for most of the month of May, while the hardcover has climbed back up into the top ten for week 20, 2014. Whatever their position, however, copies of Green’s book have been strong, selling over 1.2 million units since week 1. The buzz around The Fault in Our Stars has given boost to Green’s backlist as well, with Looking for Alaska, Paper Towns, and An Abundance of Katherines turning up in the top 20 of the Juvenile Fiction bestseller list, with combined total sales of more than half a million units among them.

As always, Nielsen Book is not only tracking book sales but also book consumer data, and through Nielsen’s Books & Consumers survey we know that John Green fans are more likely than most book buyers to be single women, with no children (often living with parents), and from the Northeast. They are highly socially connected, and are especially high participants in Goodreads, Twitter and Pinterest.

Veronica Roth readers are quite similar in gender and age, but more urban, more likely to be better educated and professionals (lawyers, doctors).

These are just snippets of the consumer and market research Nielsen Book offers. Blending consumer insights with point-of-sale data provides Nielsen clients with the most comprehensive book industry information available today. We look forward to sharing more of this data in the weeks and months ahead.

Both album and track sales were down in May over the previous month, with overall album sales down 2.3%. The “Frozen” soundtrack started the month at No. 1, following two straight months at the top of the chart. The month continued with three different releases debuting at the top: the “Now 50” collection and much anticipated releases from the Black Keys and Coldplay, whose “Ghost Stories” scored the highest one week sales total for an album so far this year. Digital track sales were down 5.1% over April. Ariana Grande’s “Problem” spent three weeks at number one and has sold over 1.2 million downloads in its four weeks of release. Iggy Azalea, who is a featured artist on “Problem,” also landed her first No. 1 download as “Fancy” topped the chart this week. “Fancy” has also spent the last two weeks at the top of the Weekly Streaming chart, ending Pharrell Williams’ long run at No. 1. Luke Bryan continues his great chart run, spending the last two weeks at the top of the Country airplay chart while Brantley Gilbert debuted at the top of the Country albums chart this week with the second best one week sales total for the genre so far in 2014.

In Canada, album sales edged up slightly in May, up 0.1%, as four different releases held the No. 1 spot on the Top Albums chart; the “Frozen” soundtrack, Sarah McLachlan, Black Keys and Coldplay, who scored the biggest one week sales total so far in 2014. A total of 11 albums from Canadian artists debuted in the top ten on the Top Albums chart during the month of May. Track sales in May were down 5.5% over the previous month. As in the U.S., Ariana Grande and Iggy Azalea have both spent time at the top of the Digital Songs chart with John Legend’s mega-hit “All Of Us” sandwiched in between for two weeks.


Retail disc sales for 2014 through week ending May 25, 2014 were down just -6% compared to year ago. Blu-ray disc and Blu-ray/DVD combo sales are up 1%. Disney’s Frozen continues to be the top title of 2014 to-date. It has also been the top title of the week for 5 of the past 6 weeks. Other animated titles continue to be best-sellers as Despicable Me 2, Cloudy With A Chance Of Meatballs 2, Pirate Fairy, and Jungle Book also landed in the top ten chart of 2014 to-date. As a result, Animated disc sales are up 17% for the year. Sequels made up half of the top ten chart of the year to-date with titles like Hunger Games: Catching Fire, Hobbit: Desolation of Smaug, Thor: The Dark World, Despicable Me 2, and Cloudy With A Chance of Meatballs 2.

Nut Job and Frozen were among the top titles in May, which helped support the 24% growth of the Family genre in the month. Also included in the May top titles is the Science Fiction title I, Frankenstein. Science Fiction genre disc sales were flat in May, but are up 14% for the year to-date. Amazing Spider-Man (rel. 11/09/2012) was in the weekly top ten charts during the first two weeks of May. This coincided with the box office release of Amazing Spider-Man 2 early in the month.

PAW Patrol, an animated TV title, released this month and quickly became the Top TV Title of May. However, Game of Thrones continues to have a strong presence with 3 titles in the top ten TV chart of the month. Also, Game of Thrones: Complete Third Season is the top TV title of 2014 to-date.

APRIL 2014

The games market kept pace with recent months with another 15 titles tracked in Nielsen’s VGT for retail release in April. With the summer blockbuster movie season fast approaching, The Amazing Spider-Man 2 swings into action on video game consoles everywhere this month. And another action/adventure movie received the LEGO treatment in the release of LEGO The Hobbit.

As if the open worlds presented in Oblivion and Skyrim weren’t big enough, fans of the Elder Scroll series can now explore the world of Tamriel in a persistent MMO environment via The Elder Scrolls Online. And 3DS gamers who enjoy the Simulation / Management genre can now visit Mama once again with Gardening Mama 2: Forest Friends.

Sports fans have a lot to celebrate this month. April saw the release of MLB 14: The Show for the PlayStation platforms, which is now the only MLB game in town with the departure of the MLB 2K series. The 2014 World Cup doesn’t start until June, but the arrival of 2014 FIFA World Cup Brazil this month allows gamers to start warming up now for the big event. And Xbox One gamers can now compete in a variety of extreme sporting events in the comfort of their living room, with the 8th Gen extension of Microsoft’s series Kinect Sports: Rivals.

While there were not any entirely new brands introduced this month, there were two new video game titles based on popular franchises for kids. Nintendo released Disney Magical World exclusively for 3DS, allowing gamers to live out a fantasy life in the World of Disney. And gamers on the Wii U can now go kart racing with their favorite Sanrio characters in Hello Kitty Kruisers.

Through May 18, 2014, sales tracked through the Nielsen BookScan US measurement service were strong – erasing the 3.7 overall year-over-year decline reported through Q1 2014.

Traditional book retailer business grew, while the Mass Merchandiser distribution channel went from 12% down to 3% down since the end of March.

Adult Fiction continues its downward trend (-13% across the board) even though small gains are being reported from the Graphic Novel and Western categories. Adult Non-Fiction is flat, while Juvenile book sales continue to set the pace for the industry – up 13% YTD.

Below is a list of the overall Nielsen top sellers through Week 20, 2013:



Author Spotlight

Fittingly, this month’s spotlight is on John Green and Veronica Roth, who have been dominating the Nielsen Juvenile Fiction bestseller charts (tying up five of the top ten overall bestseller slots). This should be no surprise, seeing as they’ve been dominating Hollywood this spring as well. Roth’s Divergent made a strong showing at the box office after its March 21st opening, and her trilogy’s sales reflect that, with Insurgent, Divergent, and Allegiant holding steady at spots 4, 5, and 6 respectively for week 25; all told, this series has sold over 2.6 million copies this year. Sales of Green’s The Fault in Our Stars reflects similar enthusiasm for the upcoming film adaptation, which opens nationwide on June 6th. The original paperback and movie tie-in paperback have been sitting at positions 1 and 2 for most of the month of May, while the hardcover has climbed back up into the top ten for week 20, 2014. Whatever their position, however, copies of Green’s book have been strong, selling over 1.2 million units since week 1. The buzz around The Fault in Our Stars has given boost to Green’s backlist as well, with Looking for Alaska, Paper Towns, and An Abundance of Katherines turning up in the top 20 of the Juvenile Fiction bestseller list, with combined total sales of more than half a million units among them.

As always, Nielsen Book is not only tracking book sales but also book consumer data, and through Nielsen’s Books & Consumers survey we know that John Green fans are more likely than most book buyers to be single women, with no children (often living with parents), and from the Northeast. They are highly socially connected, and are especially high participants in Goodreads, Twitter and Pinterest.

Veronica Roth readers are quite similar in gender and age, but more urban, more likely to be better educated and professionals (lawyers, doctors).

These are just snippets of the consumer and market research Nielsen Book offers. Blending consumer insights with point-of-sale data provides Nielsen clients with the most comprehensive book industry information available today. We look forward to sharing more of this data in the weeks and months ahead.

Both album and track sales were down in May over the previous month, with overall album sales down 2.3%. The “Frozen” soundtrack started the month at No. 1, following two straight months at the top of the chart. The month continued with three different releases debuting at the top: the “Now 50” collection and much anticipated releases from the Black Keys and Coldplay, whose “Ghost Stories” scored the highest one week sales total for an album so far this year. Digital track sales were down 5.1% over April. Ariana Grande’s “Problem” spent three weeks at number one and has sold over 1.2 million downloads in its four weeks of release. Iggy Azalea, who is a featured artist on “Problem,” also landed her first No. 1 download as “Fancy” topped the chart this week. “Fancy” has also spent the last two weeks at the top of the Weekly Streaming chart, ending Pharrell Williams’ long run at No. 1. Luke Bryan continues his great chart run, spending the last two weeks at the top of the Country airplay chart while Brantley Gilbert debuted at the top of the Country albums chart this week with the second best one week sales total for the genre so far in 2014.

In Canada, album sales edged up slightly in May, up 0.1%, as four different releases held the No. 1 spot on the Top Albums chart; the “Frozen” soundtrack, Sarah McLachlan, Black Keys and Coldplay, who scored the biggest one week sales total so far in 2014. A total of 11 albums from Canadian artists debuted in the top ten on the Top Albums chart during the month of May. Track sales in May were down 5.5% over the previous month. As in the U.S., Ariana Grande and Iggy Azalea have both spent time at the top of the Digital Songs chart with John Legend’s mega-hit “All Of Us” sandwiched in between for two weeks.

Retail disc sales for 2014 through week ending May 25, 2014 were down just -6% compared to year ago. Blu-ray disc and Blu-ray/DVD combo sales are up 1%. Disney’s Frozen continues to be the top title of 2014 to-date. It has also been the top title of the week for 5 of the past 6 weeks. Other animated titles continue to be best-sellers as Despicable Me 2, Cloudy With A Chance Of Meatballs 2, Pirate Fairy, and Jungle Book also landed in the top ten chart of 2014 to-date. As a result, Animated disc sales are up 17% for the year. Sequels made up half of the top ten chart of the year to-date with titles like Hunger Games: Catching Fire, Hobbit: Desolation of Smaug, Thor: The Dark World, Despicable Me 2, and Cloudy With A Chance of Meatballs 2. Nut Job and Frozen were among the top titles in May, which helped support the 24% growth of the Family genre in the month. Also included in the May top titles is the Science Fiction title I, Frankenstein. Science Fiction genre disc sales were flat in May, but are up 14% for the year to-date. Amazing Spider-Man (rel. 11/09/2012) was in the weekly top ten charts during the first two weeks of May. This coincided with the box office release of Amazing Spider-Man 2 early in the month. PAW Patrol, an animated TV title, released this month and quickly became the Top TV Title of May. However, Game of Thrones continues to have a strong presence with 3 titles in the top ten TV chart of the month. Also, Game of Thrones: Complete Third Season is the top TV title of 2014 to-date.

anonymous asked:

HLOY SHI T THIS WHOLE TMIE I'VE BEEN FOLLOWIGN YOH I NEVR MADE TH E CONNECTIKN BTWEEN PHAN SOMG EDEN AND TUMBLR EDEN I'VE BEEN A FAN OF THE SAME EDEN INSTEAD OF 2 DIFFERNET ONES THIS IS MNOUMENTAL

Eden-seption

it’s so surreal i was literally living in britain when torchwood was being filmed and iw as completely unaware. im pretty sure we visited cardiff often enough (esp since we moved around that time) that i wouldve been in cardiff at the same tmie that torchwood was occuring and i had no idea

anonymous asked:

(╯°□°)╯︵ ┻━┻

(╯°□°)╯︵ ┻━┻: what are your thoughts on school?

omg this confused me so much cus i thought these were 3 separate emojis

i like school but i dont?? i like learning and some classes but i have trouble getting things in and focusing (shoutout to my mental health!!) and im bad w time maagement and ? idk i did alright until high school and made the mistake of going to a catholic prep school which i couldnt handle! and now im doing online school which would be good i like it a lot cus i can deal my tmie the way i want i can spend more time on things that im struggling with and i can skip other stuff and i only have to do busy work if i want to i only have to turn in quizzes and writing assignemtns and tests and larger projects except i only had 2 hours a day to work in rehab fro the first two months of it so i got behind and now i cant stick with their schedule and im trying to finish school and i have a two week extension and im not gna make it im gna fail oh my god! im sorry ive been stresed abt this and i could go on and on oh my godd i havent gotte n anythign done and im trying to do it now aahh alrightalright

in general i think school can be cool but in general it doesnt meet the needs of it’s students and is focused less on taking people to their potential and more on conforming and meeting standards and tends to be ableist and such in how they run things and im not very good at articulating this kind of stuff but yeah