More elasticity than a rubber band

first_imgDear Editor,The laws governing the Georgetown Municipality seem to have more elasticity than a rubber band.Throughout the public service, officials from the top to the bottom are mandated to take their vacation leave in a timely and structured manner. They are not allowed to carry forward their leave nor be paid in lieu of leave. At City Hall, several persons have not taken leave in years and are either stacking it up or are being paid for it.In other municipalities around the country, elections for Mayors, Deputy Mayors, chairpersons and deputy chairpersons are done at the end of each calendar year. At the Georgetown Municipality, the election for these posts seem to be left up to the whims and fancies of the administrators.At the City Council, they practice a do as I say and not as I do policy, whereby they condemn persons’ buildings that are in a state of disrepair, whilst they sit in the derelict City Hall building pontificating without even trying to fix it. The close eating houses, butcheries, etc for unsanitary conditions while ALL of the municipal markets are rat infested, dirty and with leaking roofs. They demand that property owners pay up their rates on time and punish those who are delinquent whilst they owe billions of dollars to the Guyana Revenue Authority, the National Insurance Scheme, the Guyana Power and Light, the Guyana Water Inc, etc, for years now and are not paying up one red cent.They make their workers wait well beyond the prescribed time to be paid, cause them to occupy neglected decrepit offices while they attempt to construct luxury homes for themselves in upscale neighbourhoods. The fail to provide basic Police training for their Constables whilst instead taking off to distant luxury resorts for special retreats.It definitely seems to be ‘different strokes for different folks’ at City Hall. How else could one explain whilst several low-ranking persons received penalties for their dereliction in relation to the juvenile sex scandal case that rocked the city, nothing was done to reprove a senior member of the Engineer’s Department and an officer from the Human Resource Department when they were found showing considerable sexual dexterity below a boardroom table in a conference room?Sincerely,Sean Leviuslast_img read more

Group of citizens raise concerns about Site C employment statistics in open letter to Premier

first_img– Advertisement -In their letter, the group of 114 make 10 recommendations to Premier Horgan when it comes to getting more detailed employment statistics from Hydro on the Site C project. Those 10 recommendations are:Ask BC Hydro to release pertinent sections of Contractors’ contracts regarding criteria for job data collection especially with regard to how the data is collected and the definition of what time equivalent constitutes a ‘job’.Ask BC Hydro to determine the time equivalency of various professional and semi-professional jobs, and whether the Site C work is part of a larger portfolio.Ask BC Hydro to provide full-time employees on the “non-construction” numbers or verify that they are indeed occasional employees to the project and whether they would retain full-time employment in their companies if Site C was terminated.Ask BC Hydro to identify how many jobs in doing public infrastructure work are reported as Site C jobs.Ask BC Hydro to clarify the actual job functions and FTE details of the “worker accommodation and services…office staff…and supplies” categories.Ask BC Hydro to explain the employee days they are documenting with this number.Ask BC Hydro to verify the number of actual “construction” workforce in August 2017, in all job classifications so that the public can get a clearer picture of worker numbers. And further, would BC Hydro explain the discrepancies in numbers.Ask BC Hydro for its health and safety emergency plan given that it is not up-to-date on employment figures.Ask BC Hydro to explain the criteria that reporting contractors use to provide the ‘primary residence’ estimate.Ask if BC Hydro or the government has undertaken a study or estimate of how many workers who were previously employed at Site C have left the area, compared to those who remained and transitioned to another industry.The full letter can be found below. FORT ST. JOHN, B.C. — 114 citizens from across the province, including residents of Fort St. John, have sent an open letter to B.C. Premier John Horgan raising their concerns with BC Hydro’s employment statistics for the Site C dam project.In the letter, the group asks Premier Horgan to get Hydro to provide more detailed human resources data from Site C than is currently being offered by the Crown Corporation, with the reasoning that those statistics lack public verification. BC Hydro is required to publish monthly jobs numbers for the project, including the percentage of workers that are primary residents of B.C.However Hydro has said that the monthly jobs numbers are cumulative. That means that even if a worker is laid off during a given month, they will still be indicated as an employee in that month’s employment statistics.“We think a better energy plan would produce good paying jobs close to home in communities throughout BC,” the letter reads. “Keeping life affordable under the circumstances means limiting rate increases to only those made necessary by cancellation of the project.We also recognize and applaud your government’s commitment to improving public infrastructure and creating tens of thousands of construction jobs around BC.It is against this backdrop that we have become increasingly alarmed about the position of some members of the Legislative Assembly, the ICBA (Independent Contractors Business Association), CLAC (the Christian Labour Association of Canada) and some in the media who persist in making Site C about jobs.”Advertisementlast_img read more

Torrance grad begins her journey as a Georgetown Hoya

first_imgI, along with 14 other diverse individuals from across the nation and the world, engaged in thought-provoking dialogues about what it means to be Asian, black, Caucasian, Jewish and Latino. No topic was off-limits, whether it be affirmative action, religion, racism, sexism or, surprisingly, politics. The program left me physically and mentally exhausted, as it lasted from 8a.m. to midnight for seven days. But more than that, it caused me to completely alter the way in which I see diversity and people. We also touched on the Lesbian Gay Bisexual Transgender Queer or Questioning (LGBTQ) community, with a representative from the Human Rights Campaign. I learned about the origins of the LGBTQ flag, the privileges of heterosexual couples that are not given to gay couples, and the etymology of derogatory words and phrases used to categorize the LGBTQ community. Calling someone a “bundle of sticks,” for instance, is not right. My favorite part of YLEAD was a historical tour of Washington, D.C., where I got the chance to see all four quadrants of D.C. – not just the bubble of Georgetown that encompasses the Georgetown area, as well as Capitol Hill and the National Mall. This is where I discovered the meaning of gentrification. Gentrification is where the vitalization of run-down urban areas by the middle class results in the displacement of lower-income people. Editor’s note: With the start of the new school year comes the start of a new Freshman column. This time around, we’ll follow two students as they go through the ups and downs, excitement and anxiety, of their first year of college. Lana Buu, a Torrance High School graduate attending Georgetown University in Washington, D.C., will start things off. She’ll alternate weekly with Patti Sponaugle, another Torrance High grad, who is attending Kenyon College in Gambier, Ohio. As I arrived at Georgetown University, at 37th and Ostreets, and walked past the school’s famous Healy Gates, I felt a sense of adventure surging through me. I got to the Washington, D.C., campus a week earlier than the rest of the incoming Class of 2011 because I was accepted into the Young Leaders in Education About Diversity program. YLEAD is known among Georgetown students for producing up-and-coming leaders in organizations such as the Black Student Alliance and the NAACP. YLEADers want to make an impact in the community. By doing so they have earned prestigious awards and have been accepted into prominent academic institutions such as Oxford. I noticed it in the subtleties of each district. The road became bumpy as our knowledgeable tour guide, Dominique, a D.C. native and Georgetown student, pointed out that in the forgotten places of D.C. one can see many liquor stores, churches and “Checks Cashed” locations, but one can hardly spot a single grocery store. As YLEAD came to a close, my fellow Hoyas and I moved into our dorms. My dorm, Harbin Hall, was home to former President Clinton during his second year at the Hilltop. And George Tenet, former CIA director, was a resident adviser on the seventh floor. Georgetown traditions are ingrained in the minds and hearts of every Hoya. So some friends and I decided it was a good idea to cool off from the August heat by swimming in the Dahlgren Fountain fully clothed at 2 a.m. Other traditions, such as New Student Convocation, are unlike those of any other university. The convocation serves as a formal induction of new students – freshmen and transfers alike – to the Georgetown community. At the New Student Convocation ceremony, I was ushered into the McDonough Gym with a black graduation gown folded on my left arm. We were divided into our respective schools (Walsh School of Foreign Service – the one I’m in; McDonough School of Business; the School of Nursing and Health Studies; and Georgetown College) and recited, in unison, the Georgetown student pledge. After the pledge, the students were instructed to don their gowns. We were told these gowns were to be our graduation gowns and are a symbol of Georgetown’s commitment to each student’s academic, social and spiritual education. In my short 10 days on the Hilltop I have learned a lot about myself, my strengths and my weaknesses. And as I start to embark on my path, I find myself in deep introspection about who I am and what I want from my Georgetown experience. Lana Buu is a Torrance High School graduate writing about her freshman year at Georgetown University.160Want local news?Sign up for the Localist and stay informed Something went wrong. Please try again.subscribeCongratulations! You’re all set!last_img read more

England’s strangest call-ups? The 10 worst ever Three Lions players ranked

first_img 10 10 Playing for your country is arguably the pinnacle of any footballers career, but playing just once is a record which always seems a bit embarrassing.England managers have called up their fair share of strange players, ranging from average second tier strikers to midfielders who have spoken out about the Three Lions’ regime.But who are the worst to have ever pulled on the hallowed white shirt?Click the right arrow, above, to see talkSPORT’s collection of England’s strangest call-ups – ranked first to worst… 1. Gavin McCann (v Spain, February 2001) – Yes, this is not a wind up. Gavin McCann DID actually play for England. The Gavin McCann no one will ever remember considering his completely average career with the likes of Aston Villa, Bolton and Sunderland – where he was playing when he made played for the Three Lions in 2001. And somehow, McCann was part of a side which beat Spain, coming on at half-time as Sven-Goran Eriksson’s reign as manager got off to a perfect start. Not perfect for McCann though, as he never played for his country again. 10 7. David Nugent (v Andorra, March 2007) – Somehow still a Premier League player with Middlesbrough, Nugent remains one of the strangest England internationals of recent times. Coming through a season in which the striker netted only 15 goals for Preston in the Championship, Steve McClaren thought it wise to select him for a Euro 2008 qualifying match. Although he scored in the 3-0 win – stealing a goal from Jermain Defoe in the process, it was not a surprise his England career ended with the record of one cap/one goal. And it was a goal I could have scored! 8. Neil Ruddock (v Nigeria, November 1994) – A year after signing for Liverpool in 1993, Razor Ruddock won his one and only England cap, playing against Nigeria under Terry Venables. A solid and rather menacing defender, it was probably his playing style and, er, ‘extracurricular activities’ which prevented him adding to his tally and likely taking part in Euro 96. 5. Paul Konchesky (v Australia, February 2003) – A middling Premier League defender who was hopelessly exposed following his move to Liverpool, Konchesky somehow earned TWO caps for England. Playing well for Charlton in 2003 he came on as a half-time substitute against Australia, and although poor, Sven-Goran Eriksson decided to recall him two years later to play against Argentina. 10 10 6. Anthony Gardner (v Sweden, March 2004) – Considering England’s squad for this match included John Terry, Jonathan Woodgate and Gareth Southgate, and could have included Ledley King, Gardner should count his stars lucky he managed to play 45 minutes of a loss to Sweden. Zlatan Ibrahimovic, obviously, netted the only goal of the game, and Gardner failed to ever recapture the form which he showed that season for Spurs. 10. Joey Barton (v Spain, February 2007) – Critical of the England players who released autobiographies in the wake of failure at the 2006 World Cup, it was a surprise when Steve McClaren decided to call Barton up for a friendly clash against Spain. Despite solid form for Manchester City, Barton was already extremely outspoken, and even though he replaced Frank Lampard in the match, his following actions – including the charge for assaulting Ousmane Dabo – effectively ended his England career. Click the right arrow to see the remaining players 4. Richard Wright (v Malta, June 2003) – Another player who somehow managed to make two appearances for England, Wright’s is even more surprising than Konchesky seeing as he embarrassed himself on his debut, conceding two penalties against Malta. He was part of the England squad at Euro 2000 – not playing, thankfully – and earned a second cap the following year, playing 45 minutes of a 2-0 loss to the Netherlands. 10 10 10 2. Michael Ricketts (v the Netherlands, February 2002) – Unlike most on this list, Ricketts came into his only appearance for the Three Lions actually deserving of a call-up, having been racking up goals at an impressive rate for Bolton. He hit 24 goals in 2000/01, winning promotion for the First Division, and followed that up with 15 goals in the Premiership by February 2002. But then he played 45 embarrassing minutes for England, failing to score, and didn’t hit the back of the net for Bolton again all season. Ricketts scored just 11 times across the next three seasons before dropping back down to the Championship – and starting his rapid descent into footballing obscurity. 9. Kevin Davies (v Montenegro, October 2010) – Davies became the oldest England debutant for 60 years when he replaced Peter Crouch in a Euro 2012 qualifier against Montenegro, and in true Davies’ fashion he failed to score – but picked up a yellow card. He had come off the back of his best season in front of goal, netting 12 times for Bolton, but at the age of 33 it was clear he was hardly a long-term option for the Three Lions. He was still better than a modern-day Wayne Rooney though… 3. Jay Bothroyd (v France, November 2010) – The fact that Bothroyd played for England just six years ago, and is currently playing in the J1 League is telling that he should never have worn the famous Three Lions. However, a 2-1 friendly defeat to France in November 2010 saw the striker make his one and only appearance for his country, having scored only 12 Championship goals the season before. The other forwards in that squad were Peter Crouch and Andy Carroll, whilst Scott Loach was on the bench. A worrying time for England, really. 10 10last_img read more

‘If John Stones was a stone, I’d pick him up and throw him away’ – Fans reaction

first_img1 Stones was at fault again as Nathan Redmond gave the Saints the lead John Stones has had a pretty miserable week as miserable weeks go.The £47.5 million man suffered a nightmare performance during his side’s 4-0 defeat to Barcelona on Wednesday night, where he missed a free header, gifted Lionel Messi his hat-trick strike, and was turned inside out by Neymar for Barca’s fourth of the night.And it did not get any better for Stones on his return to Premier League action.The 22-year-old was building the play up from the back as usual before deciding to test fit again Vincent Kompany’s anticipation skills.The elegant defender was at fault again though as Nathan Redmond nipped in to give the Saints the lead.If that wasn’t bad enough, Stones celebrated like a man who righted his wrong when he found the net minutes later – before he was flagged offside.Fans took to social media to react to yet another calamity moment from England’s most expensive defender in history.last_img read more

Cops stop John Mark Karr, ‘GMA’ TV crew near school

first_img AD Quality Auto 360p 720p 1080p Top articles1/5READ MOREThe joys and headaches of holiday travel: John PhillipsKarr was giving the ABC producers a tour of the neighborhood where he used to live and work when he suddenly exited the limo and approached the school, said Jeffrey Schneider, senior vice president of ABC News. “His behavior gave us serious pause, and ABC decided not to proceed with the interview,” Schneider said. Karr was released from jail Thursday after Sonoma County authorities lost critical evidence in a 5-year-old child pornography case. He was released a little more than a month after DNA evidence cleared him of suspicion in the slaying of child beauty queen JonBenet. SAN FRANCISCO – John Mark Karr, the one-time suspect in the slaying of JonBenet Ramsey, was briefly stopped by police on Friday when the former schoolteacher was spotted wandering near the classrooms of a school where he briefly worked years ago. Sgt. Steve Mannina said a limousine carrying Karr, 41, and two producers from ABC’s “Good Morning America” show was stopped shortly after the reported sighting. Police took the names of Karr and the producers, but no one was arrested because no laws were broken. Police said an employee of the Convent of the Sacred Heart school in San Francisco, where Karr worked briefly as a teacher’s aide in 2000, called police when she spotted Karr near the school. “He stepped out of the limo and stood (outside the school) for two or three minutes, walked down the sidewalk, went up to the door and looked in the window,” Mannina said of Karr. 160Want local news?Sign up for the Localist and stay informed Something went wrong. Please try again.subscribeCongratulations! You’re all set!last_img read more

Birmingham end winless run with 1-0 victory against Sheffield Wednesday

first_imgIsaac Vassell’s goal ended Birmingham’s winless run as they clinched a 1-0 home victory against Sheffield Wednesday.The substitute earned caretaker manager Lee Carsley his first win in charge as the hosts won their first game in nine outings.It failed to lift the Blues out of the Sky Bet Championship relegation zone, and they sit in the bottom three on goal difference while inconsistent Wednesday remain in mid-table.A low-quality game looked destined to end goalless, especially when Gary Hooper’s close-range strike for Wednesday was disallowed for offside, before Vassell grabbed the 76th-minute winner.It further boosted Birmingham, who sacked Harry Redknapp earlier this month, after Carsley had bought the club time in their search for a new boss following Saturday’s 1-1 draw at Derby.But Birmingham initially struggled to impose themselves at home.It was a turgid opening and it took until the 22nd minute to see a clear chance when Wednesday’s Adam Reach should have done better than to plant a free header straight at Tomasz Kuszczak.Before then David Davis shot straight at Joe Wildsmith from distance but the hosts should have taken the lead after 29 minutes.Wednesday failed to deal with Davis’ cross and when the ball fell to Jeremie Boga, the Chelsea loanee blazed over the bar from eight yards.The miss underpinned the lack of quality at St Andrew’s, although Craig Gardner had to hook away a goal-bound header from Tom Lees just before the break.Birmingham’s finishing after the re-start initially failed to improve either as Cheikh Ndoye fired over the bar from 10 yards with just Wildsmith to beat.Wednesday then thought they had grabbed the lead after 71 minutes when Hooper tapped in Morgan Fox’s cross but it was ruled out by the linesman’s flag.However, five minutes later Vassell’s first goal for Birmingham since his summer move from Luton won it when he fired in Jacques Maghoma’s cross at the far post.Lucas Joao, Barry Bannan and Kieran Lee all had chances to level late on but Birmingham survived. 1 Birmingham caretaker Lee Carsley last_img read more

How Suarez and Liverpool stars reacted when Rodgers produced ‘the envelopes’

first_img Check out all the live commentaries coming up across the talkSPORT network this week Getty Images – Getty Brendan Rodgers returns to Anfield on Saturday for the first time since he was sacked as Liverpool boss in 2015.The Leicester manager, who spent more than three years with the Merseyside club from 2012 and 2015, is looking to do what no manager has done this season and claim at least a point against the European champions. Birmingham vs Middlesbrough (Friday, 7:45pm) – talkSPORT 2Brighton vs Tottenham (Saturday, 12:30pm) – talkSPORTFulham vs Charlton (Saturday, 12:30pm) – talkSPORT 2Burnley vs Everton (Saturday, 3pm) – talkSPORT 2West Ham vs Crystal Palace (Saturday, 5:30pm) – talkSPORT Getty Images – Getty Suarez has opened up on his time with Rodgers at Anfield Rodgers is now enjoying life as Leicester boss 2 A new style meant Andy Carroll and Charlie Adam were moved on, with midfielder Joe Allen following Rodgers from Swansea.“Brendan described Joe as the ‘Welsh Xavi’; it didn’t quite work that way, and that’s some tag to have, but Joe was especially brilliant the first 10 games or so and I thought he was an excellent signing.“Brendan knew him really well from their time at Swansea and Joe’s first few games were fantastic. He was very good with the ball, he fitted the philosophy perfectly and defensively he was exceptional; above all, he understood the movements Brendan wanted straight away, while Stevie [Gerrard] and Jordan Henderson began a process of adapting to his style.” The 46-year-old came as close as any Reds manager in the 24 years before him to winning the club’s first ever Premier League title, having eventually been pipped to the crown by Manchester City in the 2013/14 campaign.And writing in his 2015 autobiography, Luis Suarez shed some light on his time playing under Rodgers, including his infamous ‘envelopes’ tactic.In 2012, the Northern Irishman showed his Liverpool players three envelopes and claimed inside were the names of those who would let the team down that season. It was a tactic designed to motivate them to play well for the year ahead.Suarez explained he and his team-mates found it quite funny.“There was a group of us sitting there, and Glen Johnson came over and said: ‘I know who’s in the envelopes. I know what’s written on all three pieces of paper.’ Who? What?”Johnson teased Jose Enrique that it was him, though Suarez said they never did find out who the so-called Judas was.“It was unusual and I must admit that for a moment I did think: “How can you think before the season has even started that there are three people who are going to let you down?”Regardless, Suarez had a lot of respect for the Leicester manager, who spoke to him in Spanish in their first meeting to explain how his Liverpool side were going to play. 2 LIVE on talkSPORT Suarez, who later linked up with the real Xavi when he joined Barcelona in 2014, liked the ideas Rodgers was trying to put across, though he only lasted another year before being replaced by Jurgen Klopp.“[Rodgers] was interested in Spain, he had studied there, and what he’d learned there was at the heart of our style of play: passing, pressuring high, quick movement, arriving into the area rather than standing there waiting for it, coming inside from wide positions.“The way he coached us during my time there was impressive and I am sure that the methods I enjoyed and found so effective will continue to be employed. Everything Brendan does is built towards perfecting the mechanics of football and making adjustments for the next game or to fulfil a particular objective.”last_img read more

Modise named Footballer of the Year

first_img26 May 2008The inaugural Premier Soccer League Awards were hosted at the Theatre on the Track in Kyalami on Sunday evening, with Teko Modise walking away with the biggest honour and the biggest cheque after he was named Footballer of the Year, for which he pocketed a cool R250 000.Although he had a good season, Modise’s award was somewhat surprising when one considers that his club, Orlando Pirates, finished only eighth in the 16-team PSL, and lost in the first round of the Telkom Knockout Cup and the Nedbank Cup; usually individual awards are strongly tied to the success of teams.With a new look at the top end of the PSL table – after SuperSport United, Ajax Cape Town, and Santos finished 1-2-3 – the expectation was that the winner of Footballer of the Year would come from one of those clubs. Yet Modise, who netted four goals in the league, walked away with the award, which was determined by a vote of PSL coaches.SurprisedHe admitted to the PSL website that the award had surprised even him: “I didn’t expect to win anything major. The other nominees for the award were brilliant,” he said.“It was a tough season. I played lots of games for the club and the national side. I thought, at some stage through the season, that I’d burn out.”Kaizer Chiefs finished sixth in the league, but had the stingiest defence, allowing only 20 goals in their 30 games. Goalkeeper Itumeleng Khune played a big role in the Amakhosi’s defensive prowess and his contribution was recognised by his fellow professionals when he was named the Players’ Player of the Year, as well as Goalkeeper of the year. The awards were worth R150 000 and R50 000 respectively.Big impactSuperSport United winger Elias ‘Dominguez’ Pelembe was named the Absa Premiership Player of the Year, which was worth R150 000. The Mozambican certainly made a big impact in his first season in the PSL.Matsatsantsa’s league success was further reflected in the awarding of the Coach of the Year award to their coach, Gavin Hunt. It marked the second time Hunt has won the award. He previously claimed it six years ago with Black Leopards.United also claimed the goal of the season, worth R50 000, with the award going the way of defender Siboniso Gaxa for a stunning strike against the side’s Pretoria rivals, Mamelodi Sundowns.SurprisedMoroka Swallows’ striker James Chamanga received R14 000 for finishing as the top goal scorer in the PSL, which won him the Lesley Manyathela Golden Boot Award. That worked out at R1 000 for each goal he scored.If he had hit just one more goal he would have reached the minimum target set by the sponsors, Absa, and been rewarded with R50 000.Chamanga’s tally was boosted by a stunning outing against against Platinum Stars in December in which he hit five goals in a 6-2 win.Chairman’s AwardThe Chairman’s Award, decided by PSL Chairman, Dr Irvin Khoza, went to Kenyan Musa Otieno for his services to football in South Africa.He joined Santos in 1997 and was a star in defence for the People’s Team as they became a powerful presence in the PSL, including winning the title in 2002. From next season he will be campaigning in the USA, playing for the Cleveland City Stars. Otieno’s award was worth R100 000.Although Mamelodi Sundowns won the Nedbank Cup, it was the team they beat in the final, Mpumalanga Black Aces, which claimed the big individual prizes.First Division winnersThe First Division club’s Vusiwe Masondo was named Player of the Tournament, which earned him a nice bonus of R100 000, while Thabang Rooi was named the Most Promising Player of the Tournament.The National First Division Player of the Year was divided into awards for the top players in the two streams, Inland and Coastal. The Inland Player of the Year went to Dynamos’ Joseph Mthombeni, while the Coastal honours went to Ikapa Sporting FC’s Kurt Lentjies.Garankuwa United’s Reason Chiloane, despite playing for the team that finished bottom of the Inland Stream, was the top goal scorer in that section of the First Division, while the position was reversed in the Coastal Stream where Fadlu Davids of table-topping Maritzburg United was the top scorer.The referee of the year award went to Ace Ncobo, while Enock Molefe was named the best assistant.Would you like to use this article in your publicationor on your website?See: Using SAinfo materiallast_img read more

Researchers discover gene behind ‘sixth sense’ in humans

first_imgA soft brush that feels like prickly thorns. A vibrating tuning fork that produces no vibration. Not being able to tell which direction body joints are moving without looking at them. Those are some of the bizarre sensations reported by a 9-year-old girl and 19-year-old woman in a new study. The duo, researchers say, shares an extremely rare genetic mutation that may shed light on a so-called “sixth sense” in humans: proprioception, or the body’s awareness of where it is in space. The new work may even explain why some of us are klutzier than others.The patients’ affliction doesn’t have a name. It was discovered by one of the study’s lead authors, pediatric neurologist Carsten Bönnemann at the National Institutes of Health (NIH) in Bethesda, Maryland, who specializes in diagnosing unknown genetic illnesses in young people. He noticed that the girl and the woman shared a suite of physical symptoms, including hips, fingers, and feet that bent at unusual angles. They also had scoliosis, an unusual curvature of the spine. And, significantly, they had difficulty walking, showed an extreme lack of coordination, and couldn’t physically feel objects against their skin.Bönnemann screened their genomes and looked for mutations that they might have in common. One in particular stood out: a catastrophic mutation in PIEZO2, a gene that has been linked to the body’s sense of touch and its ability to perform coordinated movements. At about the same time, in a “very lucky accident,” Bönnemann attended a lecture by Alexander Chesler, a neurologist also at NIH, on PIEZO2. Bönnemann invited Chesler to help study his newly identified patients.It wasn’t the disease’s rarity that so shocked Chesler when he met the girl and young woman; it was the fact that when scientists had previously knocked out PIEZO2 in mouse models, it had always proven fatal. Most assumed people couldn’t live without it, either.The researchers performed a battery of tests with the patients and a control group. When blindfolded, the patients staggered, stumbled, and fell. But with the blindfold removed, they could walk almost normally. The patients also performed a task where they moved their index finger from their nose to a target placed in front of them. Blindfolded, they failed miserably. Eyes uncovered, they did well. The researchers held the patients’ arms and moved the joints either up or down, asking them to indicate the direction. Blindfolded, they couldn’t tell which direction their joints were being moved. No blindfold, and—naturally—they could tell just by looking.Together, the tests suggested the patients totally lacked proprioception, the researchers report online today in The New England Journal of Medicine. Healthy individuals rely on the sense to perform a variety of tasks like playing the piano, shifting gears in a car, or typing on a keyboard, Bönnemann explains. Doing these things requires awareness of one’s limbs in space. The patients lacked this instinctual awareness, but were able to largely compensate for it by watching their limbs.The researchers also tested the patients’ responses to a variety of touch tests. In one, the girl and woman could not sense the vibrations of a tuning fork pressed against their skin. In another, they couldn’t feel a soft brush swept across their palms or bottoms of their feet; against hairy skin, the brush felt prickly. This struck Chesler and Bönnemann as odd because most people report the brush feels pleasant.The scientists repeated their tests with the patients strapped into an MRI machine. They found that although healthy people’s brains show activation in a region of the brain linked to experiencing physical sensation, that activation was missing in the young woman’s and the girl’s brains. Instead, when the researchers brushed the patients’ hairy skin, the two showed brain activity in a different region linked to the emotional response to touch. They couldn’t physically feel the brush, Chesler explains, but they experienced something like an emotional reaction to its touch.Finally, the researchers had the patients hold a device that slowly became painfully hot or cold. Surprisingly, the patients were just as good as the control group at determining changes in temperature and feeling pain.Tallying up the test results, Chesler and Bönnemann determined that the PIEZO2 gene is likely critical for proprioception and sensing skin touch, but not for sensing temperature or pain. Though their study’s sample size of two is incredibly small, Chesler says he is confident the results shed light on the gene’s role in the general population. “It’s all consistent with what we’ve seen in the animal models, and it just makes sense.”There’s no telling just how rare the mutation is, Bönnemann adds, but now that he and Chesler have identified both its physical and genetic signature, he suspects they’ll locate more people with the disorder.How PIEZO2 might relate to the patients’ skeletal deformities is less clear. One possibility is that the proteins controlled by the gene play a key, as-yet unknown, role in development. Another, Chesler and Bönnemann note, is that proprioception itself might be necessary for normal skeletal development. Without it, the body can’t hold a straight posture or orient its joints correctly, which could lead to abnormal skeletal development over time.“I think these claims are provocative but indeed possible,” says Ardem Patapoutian of the Scripps Research Institute in San Diego, California, who led the previous PIEZO2 mice studies. “An indirect role of touch or proprioception in bone formation is an intriguing and exciting possibility.”Another intriguing possibility? The researchers speculate that different variations of the PIEZO2 gene might contribute to whether a person is klutzy, coordinated, or something in between. “Could a finely tuned PIEZO2 gene contribute to superior athletic performance, or a poorly tuned one to clumsiness?” Bönnemann says. “I think it’s not impossible.”last_img read more