John Grigg
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Articles by John Grigg
AAV8- PDE6B with 3’UTR provides rescue of photoreceptor structure and function in the rd10 mouse model of retinitis pigmentosa
Abstract Retinitis pigmentosa (RP), a leading cause of inherited blindness, results in a subset of cases from phosphodiesterase 6 (PDE6) deficiency. We evaluated a novel gene therapy construct, AAV8-RK-PDE6B-3′UTR, in the rd10 mouse model which has a homozygous missense variant that disrupts the function of the β-subunit of PDE6.
The quality-of-life impacts and economic burden of X-linked retinitis pigmentosa caused by variants in RPGR
Abstract To estimate the health and societal costs and health-related quality of life (HRQoL) impacts of X-linked retinitis pigmentosa (XLRP) caused by RPGR. Primary data were obtained from a clinical cohort of 9 patients with X-linked retinitis pigmentosa (XLRP) and 4 caregivers. Health-related quality of life (HRQoL) impacts for patients and carers are assessed using utility values derived from the Assessment of Quality of Life–8 Dimension (AQoL-8D) instrument.
Visual Acuity Alone Is Not Enough: The Need for Multimodal Biomarkers in Dominant Optic Atrophy-Response
No abstract is available for this article. Data Availability Statement Data sharing not applicable to this article as no datasets were generated or analysed during the current study. References 1, , , and , “Visual Acuity Alone Is Not Enough: The Need for Multimodal Biomarkers in Dominant Optic Atrophy,” Clinical & Experimental Ophthalmology (2025).
Connecting cilium, stress response, and proteostasis abnormalities inform variant and therapy assessment in RPGRIP1 retinal organoids
Results We applied TruSight One Clinical Exome sequencing to examine a bioinformatic gene panel of 65 IRD genes, which identified RPGRIP1 variants in 2 patients, LCA-1 and LCA-2, with poor vision and nystagmus manifested in the first 3 months of life. Electroretinograms showed reduced responses, and poor visual acuity was consistent at last review at ages 29 and 27 years for LCA-1 and LCA-2, respectively, with ophthalmic changes characteristic of LCA (Figures S1A and S1B).
Clinical & Experimental Ophthalmology | RANZCO Journal | Wiley Online Library
EDITORIAL Phototransduction in the Genomic Era: Mechanisms, Genetics and Therapeutic Advances John R. Grigg, Corresponding Author John R. Grigg Save Sight Institute, Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia Eye Genetics Research Unit, Children's Medical Research Institute, Sydney Children's Hospitals Network, Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia Correspondence: John R.
Skin fragility disorder misdiagnosed as child abuse: a cautionary tale
Med J Aust 2024; 221 (3): . || doi: 10.5694/mja2.52369 Published online: 5 August 2024 To the Editor: We read with interest the report by Willems and colleagues1 on skin fragility disorder being misdiagnosed as child abuse, which highlights genetic cutaneous mimics of child abuse. We highlight retinal mimics of child abuse and reiterate the role of genetic testing in selected cases. The full article is accessible to AMA members and paid subscribers. Login to read more or purchase a subscription now.
Structural characterization of two full length γδTCR/CD3 complexes
Abstract The T-cell receptor (TCR)/CD3 complex plays an essential role in the immune response and is a key player in cancer immunotherapies. There are two classes of TCR/CD3 complexes, defined by their TCR chain usage (αβ or γδ). Recently reported structures have revealed the organization of the αβ TCR/CD3 complex, but similar studies regarding the γδ TCR/CD3 complex have lagged behind.
Managing the apparently blind child presenting in the first year of life: A review
1 INTRODUCTION The child who appears to not to see in the first year of life is a distressing finding for the family. Eye health professionals require a systematic approach to managing these children from both an ophthalmic perspective as well as the societal expectations. Significant anxiety accompanies this presentation partly due to the potential prospect of a lifetime of vision impairment for their child.
A multinational survey of potential participant perspectives on ocular gene therapy - Gene Therapy
Abstract Amidst rapid advancements in ocular gene therapy, understanding patient perspectives is crucial for shaping future treatment choices and research directions. This international cross-sectional survey evaluated knowledge, attitudes, and perceptions of ocular genetic therapies among potential recipients with inherited retinal diseases (IRDs).
Suspected case of benign familial fleck retina with functional loss
1 INTRODUCTION Familial Benign Fleck Retina (FBFR) (OMIM #228980) is an autosomal recessive condition caused by biallelic mutations in the gene PLA2G5 that encodes a family V phospholipase A2 which has multiple roles including signal transduction, phospholipid metabolism, and regulating phagocytosis.1-6 This rare condition has been observed predominantly in children and young adults that present with yellow to white symmetrical flecks bilaterally with otherwise normal acuity, visual fields,...
First comes faith, then comes politics
Last week, we published a selection of responses to our question, ““During our turbulent and challenging times, how does your faith, religion or spirituality inform your politics?” A few readers followed up with additional thoughts that we wanted to share. The following was submitted by John Grigg, a retired Marine. When it comes to my faith I begin with one simple statement: I'll meet you at the cross. I believe in Christ and the miracle of salvation through faith.
The electroretinogram in the genomics era: outer retinal disorders
1. Lamb TD. Why rods and cones? Eye. 2016;30:179–85. CAS PubMed Article Google Scholar 2. Cornish EE, Hendrickson AE, Provis JM. Distribution of short-wavelength-sensitive cones in human fetal and postnatal retina: early development of spatial order and density profiles. Vis Res. 2004;44:2019–26. PubMed Article Google Scholar 3. Provis JM, Penfold PL, Cornish EE, Sandercoe TM, Madigan MC. Anatomy and development of the macula: specialisation and the vulnerability to macular degeneration. Clin Exp Optom.
Fabry Disease: An Ophthalmology Perspective
1.5 hours with Assessment – Optometry Australia IoE Quality Assurance | TBC in New Zealand Fabry Disease is a rare disease that does not typically disturb visual function. However, ocular signs of corneal verticillata, spoke-like cataract, or vessel tortuosity may be key to its diagnosis. Being able to recognise the signs and symptoms of Fabry Disease, and knowing when to refer on to a physician, can help expedite diagnosis of this debilitating condition…Read and take test.
ICYMI: Flint Firebirds saga in one easy timeline
Jeff Marek discusses the Flint Firebirds situation, team owner fires coaching staff after Sunday's overtime victory, CHL Commissioner David Branch then reinstated the coaching staff. It was the wildest 18 or so hours in recent Ontario Hockey League history. The firestorm that erupted around the Flint Firebirds late Sunday night when the coaching staff was fired after a win and the players revolted captivated hockey fans across North America.
Host-Microbiota Interactions Shape Local and Systemic Inflammatory Diseases
Host-Microbiota Interactions Shape Local and Systemic Inflammatory Diseases John B. Grigg, Gregory F. Sonnenberg The Journal of Immunology January 15, 2017, 198 (2) 564-571; DOI: 10.4049/jimmunol.1601621
Host-Microbiota Interactions Shape Local and Systemic Inflammatory Diseases
Recent advances in understanding how the mammalian immune system and intestinal microbiota functionally interact have yielded novel insights for human health and disease.
WJC Power Rankings: Nine Team Canada selection camp snubs
Hockey Canada announced the selection camp roster for its 2017 World Junior Championship squad Tuesday. It’s a deep group full of top NHL Draft picks—Nolan Patrick is the only draft eligible invited, and who knows what’s going to happen with him—and could still be buttressed by players loaned to the team by their NHL clubs. Potential pros include blueliner Jakob Chychrun and winger Lawson Crouse from Arizona, Philly centre Travis Konecny and Islanders winger Anthony Beauvillier.
Powering Down: Saskatoon Blades ban cell phones
The Saskatoon Blades have done what so many parents around dinner tables and teachers in classrooms would love to do—they’ve separated teenagers from their phones. The CBC reported Sunday that the Blades are testing a policy that has players hand over their cell phones upon entering the rink for practices and games. According to Saskatoon head coach Dean Brockman, players had been relinquishing their phones about 90 minutes before games, but no more, at least for now.
CHL on Sportsnet preview: Sault Ste. Marie Greyhounds vs. Mississauga Steelheads
The CHL on Sportsnet begins tonight at 7:30 ET with the Soo Greyhouds visiting the Mississauga Steelheads. It’s the first of a 23-game regular-season slate on Sportsnet en route to the MasterCard Memorial Cup in May. Here are a few things to get you ready for tonight’s tilt. The Mississauga Steelheads are the OHL’s most disappointing team so far this season.
The eight most important trades in the OHL so far this season
On Sunday, the Erie Otters hit the jackpot when the Arizona Coyotes returned Dylan Strome to the OHL after a seven-game NHL look-see at the 19-year-old. He has averaged 1.9 points per game with Erie the past two seasons and won the OHL scoring title two years ago with 129 points.
What do you know? The London Knights are rolling. Again.
The CHL Top 10 Rankings were released Wednesday, and it seems like things are starting to normalize. After nine weeks, gone—or mostly gone—are the wild swings that have seen teams jump on and off the list. The average number of weeks spent in the rankings for this week's group is 6.2, with Rouyn-Noranda and Shawinigan having made every list and Oshawa debuting. Thanks to Prospect-Stats.com for allowing us an alternative look at the 13 teams named. The chart is sorted by points percentage.
Not much excitement in Game 5 of the Canada-Russia Series
The fifth game of the CIBC Canada-Russia series began about 20 hours after Game 4 ended. That the latter was played in Hamilton and the former 1,100 kilometres away in Chicoutimi did not bode well for Russia. I feel for this Russian team. In nine days, it’s now played five games and covered nearly 4,500 kilometres by air and ground from Prince George to Chicoutimi.
Why Eric Lindros is the most important CHL player ever
In his story about Eric Lindros’s career and legacy, Rooting for Goliath, Gare Joyce writes that he has been telling people they’re wrong about Lindros since the early 1990s; that Lindros has been misunderstood since he was 16 years old. To be a teenaged hockey star in Canada—“The Next One”—is pressure enough. To be perceived by many as a villain while being a teenage hockey star in this country would be too much for many. It was not for Eric Lindros.
Team Russia shows grit in Game 3 of the CIBC Canada Russia Series
After a tough back-to-back in the WHL to start the CIBC Canada Russia Series, a Game 2 that ended sour and a 15-hour travel day Wednesday, the barnstorming Russians (those not joining from each of the three CHL leagues), entered their first contest against Team OHL battle-tested and road weary. In what can only be called a hard-fought, physical game, Team Russia, buttressed by a handful of countrymen from the OHL, managed to grind out a 4-3, Game 3 victory in North Bay, Ont.
Why Peterborough Petes goalie Dylan Wells is the OHL's best
Quick! Who’s the OHL’s best goalie? That’s the question I asked an OHL team executive the other day. He rhymed off a few names—McNiven in Owen Sound, DiPietro in Windsor, Parsons in London, and mentioned a few more people might look to but he didn’t think were the right answer. But the first name out of his mouth was Peterborough’s Dylan Wells, which was perfect because, based on the numbers, that’s who I wanted him to say.
10 takeaways from Game 1 of the CIBC Canada-Russia Series
The first game of the CIBC Canada-Russia Series went Monday night in Prince George with Team Russia topping Team WHL 3-2 in overtime. The WHL never had a lead, but if you’re looking for a reason it didn’t put the game away, try 1-for-7 with the man advantage. But this series is less about the wins (totally assumed and definitely expected in Canada) and more about the showcase.
CHL Notebook: Goals, gaffes and a gargantuan lawsuit
The big news of the week that was in the CHL came off the ice as the ongoing saga surrounding a $180-million class action lawsuit against the league continues. An Alberta judge ordered the 42 WHL and OHL teams to open their books and reveal the numbers behind their businesses. So, for the first time, the public might actually get a good picture of the kind of revenue major-junior hockey franchises generate.
Why the Saint John Sea Dogs may need to shake things up
There’s something funny going on down east. In our pre-season QMJHL coaches poll, the Saint John Sea Dogs were picked to win the league and even got some love from coaches in the other two CHL loops as MasterCard Memorial Cup contenders. But after beginning the season ranked No. 2 in the nation, they have been steadily sliding down the rankings. Now, it's not as if Saint John is crapping the bag.
How the OHL's top-rated prospects have started the season
When NHL Central Scouting released its Players to Watch list earlier this month, 29 skaters were given an “A” rating. Roughly 20 percent of that group ply their trade in the OHL: Erie’s Ivan Lodnia, Windsor’s Gabriel Vilardi, Hamilton’s MacKenzie Entwistle and Matthew Strome, and Mississauga’s Nicolas Hague and Owen Tippett. And since we’re 20 percent of the way through the OHL campaign already, I figured the synergy was just too awesome not to check in on the O’s Big 6. So here we go.
Developments in class action lawsuit against the Canadian Hockey League
There is a potential showdown looming in the Canadian court system over Canadian Hockey League players and the money they receive—or don’t—for playing in the league. On one side are the plaintiffs, who are trying to show that the CHL is big business and that its players are actually employees and deserve to be recognized as such under Canadian labour laws.
Why the Otters are a sleeper in OHL’s Western Conference
The Canadian Hockey League released it’s Top 10 Rankings for the Week 5 on Wednesday and a familiar team appears to be working its way back up the national ladder. That comfortable old pair of jeans is none other than the Erie Otters, who CHL fans know have been terrorizing the opposition for three years, becoming the first OHL team to post back-to-back-to-back 50-win campaigns.
Did the OHL's sleeping giant just wake up?
The CHL Top 10 for Week 4 came out Wednesday with four OHL teams making the nod and another earning an honourable mention. One team a number of OHL pundits have pegged to contend for a league title this season that was deservedly missing is the Mississauga Steelheads. When the rankings came out they were scuffling along at 2-4-0-0 with a miserable goal differential of minus-9. But then a few hours later, Mississauga pasted Guelph 11-3, the most lopsided game of the young OHL season.
2016-17 CHL pre-season coaches polls
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Stamkos comes to those who wait-or, how to win your keeper league
You will never be a professional hockey player. You will never be a billionaire owner. You will never work for an NHL franchise. You might never even learn to skate. You are the majority. But you are smart. You are a visionary. You are a hockey fan. You have theories, and you want a place to test them. Welcome. I have a secret for you. I have been playing fantasy sports for 25 years. What began with pencil and paper in the school cafeteria morphed into jumping between sites and formats.
Are the Hamilton Bulldogs all smoke and mirrors?
So this week the OHL is accounting for 40 percent of the CHL’s Top 10 Rankings. The percentage of teams isn’t surprising, but one team is: the Hamilton Bulldogs. Now, it's early, and this is no knock against a team on the up side of the junior-hockey curve. The Bulldogs are already plus-14 in goals-for and against, and boast A-listed draft prospects Mackenzie Entwistle and the third Strome brother, Matthew.
Sportsnet Road Trip: In the water with Great Whites in South Africa
THERE IS NO fear like fearing for your life. And in the lead-up to jumping in the water with great whites that couldn’t fit floor to ceiling in most living rooms, you have a lot of time to ponder that fear.
A small-molecule RIG-I agonist functions to enhance vaccine protection against influenza A virus infection.
Viral sensing by RIG-I and downstream activation of antiviral defenses along with the induction of innate immune cytokines is essential from protection against influenza A virus (IAV) infection. We have identified a novel, small-molecule RIG-I agonist, KIN1148, which binds and activates RIG-I to signal the activation of IRF3 and the innate immune response. We are developing this molecule as an adjuvant to enhance vaccination against pandemic H1N1 (pH1N1) IAV.
Small molecule agonists of IRF3 activation function as influenza vaccine adjuvants by modulating the humoral and cellular anti-viral immune response
We have identified a panel of small molecule immunomodulators that activate IRF3 and induce innate immune signaling to drive an antigen-specific protective immune response against viral infections. Our lead adjuvant candidate, KIN1148, binds to retinoic acid inducible gene-I (RIG-I) and induces RIG-I signaling to drive IRF3 activation.
OHL reviewing Flint Firebirds' firing of coaches
TORONTO — The Ontario Hockey League is reviewing the Flint Firebirds’ decision to fire their coaching staff for the second time this season. The Firebirds announced Wednesday that head coach and general manager John Gruden and assistant coach Dave Karpa had been fired "effective immediately," with director of hockey operations Sergei Kharin named interim head coach. Flint fired Gruden, Karpa and assistant coach Petr Jonak — who keeps his position with the team this time around — on Nov.
Canada's world junior championship report card
Through three games, Canada has largely disappointed at the 2016 World Junior Championship. Just one regulation win in three tries and five points total is not what Canadian fans expected from their team to open the tournament. Throw away the Denmark win, that was just men against boys. When you do that, it’s 0-0-1-1 with a goal differential of minus-2. In general, it appears the team is enamoured with the extra width of the ice surface.
Bruins loan Pastrnak to Czech Republic for WJC
The 2016 World Junior Championship got an injection of star power on Boxing Day when Boston Bruins GM Don Sweeney announced that the team would make forward David Pastrnak available to the Czech Republic’s once the NHL roster freeze ends Dec. 28. The 25th pick of the 2014 NHL Draft jumped straight to the AHL last season and lit it up, scoring 28 points in 25 games.
Top junior stories of 2015: McDavid is for real
From Connor McDavid rewriting the record books and proving himself a generational talent to Canada ending its IIHF World Junior Championship drought to the Oshawa Generals winning the MasterCard Memorial Cup to a team walking out on its owner, there is no doubt that 2015 has been one to remember. Here are your top junior hockey stories of the year. 10.
CHL coaches poll: The best at everything
On the eve of the Canadian Hockey League season, Sportsnet magazine polled coaches and executives from every CHL team about their respective leagues. We asked 14 questions covering everything from best overall player to hardest hitter to who will win the MasterCard Memorial Cup. In our first installment, we ran down the results for the top forwards, hitters and NHL Draft prospects. Then we went through some team questions, including league and Memorial Cup favourites.
CHL poll: Top teams, Memorial Cup favourites
On the eve of the Canadian Hockey League season, Sportsnet magazine polled coaches and executives from every CHL team about their respective leagues. We asked 14 questions covering everything from best overall player to hardest hitter to who will win the scoring title. In our first installment, we ran down the results for the top forwards, hitters and NHL Draft prospects in each of the three leagues.
CHL poll: Top forwards, hitters, draft prospects
On the eve of the Canadian Hockey League season, Sportsnet magazine polled coaches and executives from every CHL team about their respective leagues. We asked 14 questions covering everything from best overall player to hardest hitter to MasterCard Memorial Cup favourite. We’ll be rolling out all the answers in the coming days. We begin with… Everyone knows Marner, the London Knight who lost the scoring title last season by a point and then went fourth overall to the Maple Leafs in the NHL Draft.
Back in AL East, Arencibia has no ill-will to Jays
Knowing when the time is right to move on, however, is worth more than a victory. The former Toronto catcher, now with the Baltimore Orioles, said on Wednesday at the team’s spring training facility at Ed Smith Stadium that he’s not about holding grudges against the Blue Jays, who let him walk after the 2013 season. “You just have to understand that stuff, anyone could write anything; they don’t owe me an apology, they don’t have to come and say anything,” said Arencibia.
From the archive: A long reign and a lost republic
A long reign and a lost republic The celebration of Queen Elizabeth’s sixty years on the throne coincides with the best of recent times for the British monarchy.
Rules that changed the game
The NHL is holding a research and development camp Wednesday and Thursday in Toronto featuring a number of top 2011 draft prospects who are being put through their paces by coaches Ken Hitchcock and Dave King. Those paces include a number of tinkerings with things like overtime formats, line changes and faceoff configurations.
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