Sony Digital Rapids Pixel Power calrec Sony

June 26, 2020

26/06/2020

Huntington's disease progression involves DNA damage sensor Abnormal cellular housekeeping and inflammation point to potential target for treating fatal neurodegenerative disorder.

June 26, 2020

JUPITER, FL Families afflicted by Huntington's disease face anguishing challenges. An incurable genetic disease, Huntington's symptoms usually appear after age 30, meaning parents often pass the mutant Huntington's gene to their children even grandchildren before they discover they are carriers. Striking in the prime of life, it damages movement control, mood stability and thinking before ultimately causing death. At least 30,000 people in the United States live with the diagnosis.

So far there has been no effective therapy available for the disease because we haven't yet understood the molecular mechanism, even though we know the faulty gene, says neuroscientist Srinivasa Subramaniam, PhD, associate professor at Scripps Research in Florida.

A new study from Subramaniam and his team provides new insights into the molecular events that lead to the progressive destruction of brain cells in Huntington's. The research reveals a central role for a damage-sensing enzyme called cGAS that seems to ignite a cascade of inflammation and excessive cellular housekeeping, a process called autophagy. Their study, appearing in the Proceedings of the National Academy of Sciences, raises the possibility that reducing cGAS activity in the brain may be a treatment strategy worth testing in further studies, Subramaniam says.

The lack of an effective treatment is very frustrating for patients and their families but we hope that this new study brings us closer to an understanding of the mechanism and eventual development of such a treatment, says Subramaniam, who has been doing Huntington's research for 15 years.

Subramaniam and his colleagues discovered that the cGAS enzyme is produced at abnormally high levels in Huntington's-mutant neurons from the striatum, the brain region that is hit earliest and hardest by Huntington's disease. The team also found that the high levels of cGAS in striatal Huntington's neurons drives inflammatory processes in these cells as well as a related cellular waste-removal process called autophagy. Both inflammation and autophagy were known to occur excessively or otherwise abnormally in Huntington's and have been suspected of contributing to the neuronal destruction that underlies the disease.

The cGAS enzyme has been known as a DNA sensor that switches on inflammation and autophagy when it detects damaged DNA in a cell, or foreign DNA such as from a virus. Subramaniam and colleagues found evidence in their study that striatal neurons with the Huntington's mutation often contain numerous fragments of DNA in the main part of the cell, outside the nucleus where DNA normally is housed. These DNA micronuclei, as the researchers call them, are not present in healthy striatal neurons.

The findings suggest that the Huntington's disease process is leading to the appearance of DNA micronuclei in the cytoplasm of striatal neurons; these pieces of stray DNA activate cGAS which in turn triggers abnormal, cell-harming inflammation and autophagy.

The scientists now plan to test this hypothesis by reducing cGAS activity with drug-like molecules or genetic engineering in animal models of Huntington's disease, to see if that slows the progression of the disease. If cGAS-reduction proves safe and beneficial in such models, it could point to the usefulness of treatment strategies that target that signaling pathway.

We'll need to follow up with further study, but our linking of cGAS to inflammation and autophagy in Huntington's striatal neurons suggests that it is a good target for further research as a potential Huntington's drug target, Subramaniam says.

Cyclic GMP-AMP Synthase Promotes the Inflammatory and Autophagy Responses in Huntington Disease, was written by Manish Sharma, Sumitha Rajendra Rao, Neelam Shahani, Uri Nimrod Ramirez Jarquin, and Srinivasa Subramaniam, all of Scripps Research at the time of the study.

Funding was provided by the National Institute of Neurological Disorders and Stroke (R01-NS087019-01A1, R01-NS094577-01A1), the Cure Huntington Disease Initiative (CHDI), and the Community Foundation for Palm Beach and Martin Counties.

Neuroscience Subramaniam, Srinivasa
LINK: https://www.scripps.edu/news-and-events/press-room/2020/20200626-subra...
See more stories from scripps

Europe Stories

04/07/2020

Elite Multimedia takes the show online in a Virtual Event Production Studio

Elite Multimedia takes the show online in a Virtual Event Production Studio posted: 04/07/2020 Nashville-based production provider adapts to the challenge...

04/07/2020

#NewReplayExperience webinar on Wednesday, July 15th at 10:00 CEST or 18:00 CEST

#NewReplayExperience webinar on Wednesday, July 15th at 10:00 CEST or 18:00 CEST posted: 03/07/2020 Working alongside the LSM-VIA remote, the new versatil...

04/07/2020

Techtel integrates Bluefish444 IngeSTore Server with Inflo for network control of Tasmanian Parliament's centralised SDI ingest

Techtel integrates Bluefish444 IngeSTore Server with Inflo for network control ...

04/07/2020

Meet Your Maker: Sally Baines

Welcome to the third instalment of Meet Your Maker Q&A! Meet Your Maker is a series of behind-the-scenes videos and blogs that illustrate the wide range of in-h...

04/07/2020

Far EasTone launches Ericsson-powered commercial 5G in Taiwan

Ericsson was announced as FET's RAN vendor in March this year. Since 2015, Ericsson has been the sole provider of FET's Evolved Packet Core (EPC) networ...

04/07/2020

Grundfos pursues Industry 4.0 with Ericsson and TDC NET 5G dedicated network

Under the proof of concept (PoC) partnership, Ericsson and TDC NET will deploy 5G technology at one of Grundfos' plants in Bjerringbro, Denmark, replacing w...

04/07/2020

Ericsson powers Vodafone UK Standalone 5G network at Coventry University

The Standalone 5G network will be used to trial virtual reality learning to support training for student nurses and allied health professionals. Ericsson partn...

04/07/2020

SGO introduces revolutionary Mistika 10 in an exclusive event for the Spanish-speaking Creative Community

View Larger Image [SPANISH BELOW] SGO presents an exclusive Spanish language...

04/07/2020

Nana Hughes appointed as ITV Head of Scripted Comedy

ITV has announced Nana Hughes as the newly appointed Head of Scripted Comedy. Reporting into Head of Drama, Polly Hill, Nana will be responsible for driving an...

03/07/2020

Western Balkan Members positively assess EC-funded project

Western Balkan Members positively assess EC-funded project...

03/07/2020

RT announces new RT Six One News presenter

RT News today announced that David McCullagh will join Caitriona Perry as the new permanent presenter of the RT Six One News. Journalist and Broadcaster Davi...

03/07/2020

Summer listening sorted, as RT Radio 1 announces new shows for season

RT Radio 1 has announced a selection of new and returning factual, arts, and music series to engage and entertain listeners for the summer season. New five-pa...

03/07/2020

Invitation to presentation of Net Insights Interim report January - June 2020

Net Insight appoints Chief Commercial Officer Stockholm, Sweden - With the objective to strengthen the commercial functions, Net Insight has today appointed M r...

03/07/2020

MEDIAPRO Canada selected as TV production and international media rights partner of CEBL Summer Series in 2020

MEDIAPRO Canada selected as TV production and international media rights partner...

03/07/2020

Aston Element design enters final phase

Last chance to help design Aston's new microphone As we've reported before (see here), Aston's newest microphone, the Element, is being designed...

03/07/2020

Getting Hamilton from the stage to the screen to the stream

[The cast] knew exactly what to do, said director Thomas Kail. My job was to not get in the way of that. By Dan Meier Published: July 3, 2020 [The cast...

03/07/2020

A view to the future of post production

ENVYs technical operations director Jai Cave discusses the companys forward thinking approach to post production By Contributor Published: July 3, 2020 ENV...

03/07/2020

Black Dragon Capital completes Grass Valley acquisition

Deal first announced in early February By Jenny Priestley Published: July 3, 2020 Deal first announced in early February target=_blank title=Share on Lin...

03/07/2020

The distribution model of live sports must change': Eleven Sports founder on the future of broadcasting

You should be able to get access to a football game as easily as buying an ice c...

03/07/2020

'Mixing Pop Music' video course from Samplecraze.com

How to mix and produce a commercial friendly pop mix The best way to explain how to mix a pop song is to actually mix a pop song, from start to finish! Spanni...

03/07/2020

Zoom H8 Portable Recorder unveiled

Award-winning H-series gets new flagship model Zoom's H-series of portable recorders has long been a favourite for musicians and engineers wanting to ca...

03/07/2020

Atomos and Z CAM release 5.8K and 4K ProRes RAW recording and camera control for E2 series cinema cameras

Atomos and Z CAM release 5.8K and 4K ProRes RAW recording and camera control for...

03/07/2020

Garth Brooks Returns to the Stage with Bandit Lites

Garth Brooks Returns to the Stage with Bandit Lites posted: 03/07/2020 Garth Brooks returned to the stage in true spectacular fashion, as only Garth can, in...

03/07/2020

Grass Valley Acquisition by Black Dragon Capital Completed

Grass Valley Acquisition by Black Dragon Capital Completed posted: 03/07/2020 Grass Valley announces the completion of its acquisition by private equity fir...

03/07/2020

Elation Professional presents Virtual at Home Demo series

Elation Professional presents Virtual at Home Demo series posted: 03/07/2020 With the travel, gathering and distancing restrictions that come with the C...

03/07/2020

Robe Helps Play It Forward with Oooth

Robe Helps Play It Forward with Oooth posted: 03/07/2020 Play It Forward an incendiary new live show by South African rockers Oooth - well known for the...

03/07/2020

Sony Professional Europe adds Singular.Live graphics to Virtual Production the cloud-based live production workflow

Sony Professional Europe adds Singular.Live graphics to Virtual Production the c...

03/07/2020

Nokia to unveil breakthrough innovation in data center networking #TheSwitchisOn

Nokia to unveil breakthrough innovation in data center networking #TheSwitchisOn Media Advisory Nokia to unveil breakthrough innovation in data center netwo...

03/07/2020

Princess Royal: Anne at 70

Princess Royal: Anne at 70 As HRH The Princess Royal approaches her 70th birthday, this unique 90-minute documentary brings viewers unprecedented access behind...

03/07/2020

Fujifilm is headline partner for relaunched NHS Parliamentary Awards that celebrate the nation's health and care heroes'

Fujifilm is headline partner for relaunched NHS Parliamentary Awards that celebr...

03/07/2020

South African Rapper Willy Cardiac Is a Global Star in the Making

Willy Cardiac may have just turned 19, but the rapper has been a breakout talent in South Africa for several years. And now the Pretoria-based teen has leveled ...

03/07/2020

India's Sanjeeta Bhattacharya Dabbles Freely Across Styles

Sanjeeta Bhattacharya may have studied overseas at Boston's famed Berklee College of Music, but the Delhi-based singer-songwriter is really a lifelong stude...

03/07/2020

New Spotify Premium Duo Subscription Plan Launches in 55 Markets

You love Today's Top Hits, yet your partner is obsessed with All Out 80s. So deciding who gets to play DJ at home or in the car is a constant battle. With S...

02/07/2020

In conversation with NEP, Grass Valley and Net Insight

What happens next with live broadcasting? We got together with Donald Begg from NEP UK, Boromy Ung from Grass Valley, Per Lindgren from Net Insight, Riikka Kop...

02/07/2020

The six tools to help you protect your service

As service providers and operators extend or migrate to IP environments, new challenges arise every day, notably from commercial pirates. And while they may app...

02/07/2020

The Focusrite Guide To Releasing Music: Part 1

Words: Chuck Fishman You've recorded a song. Or a few songs. Maybe it's a collection of songs - an album - now you're thinking about how to release...

02/07/2020

5 Piges viter avant de migrer vers un Cloud Public

Dans le contexte actuel, plusieurs sujets portent r flexion : le travail distance, la continuit op rationnelle ou lacc s aux contenus archiv s en libre-ser...

02/07/2020

Ericsson and Getinge partner to increase production of advanced ICU ventilators

While the majority of people who get sick from COVID-19 will not need hospital care, for the small percentage that do, advanced ICU ventilators or machines th...

02/07/2020

Ericsson 5G commercially live with MTN in South African cities

Ericsson was announced as an MTN South Africa vendor in November 2019, to deploy products and solutions spanning its radio access network (RAN) Ericsson Radio S...

02/07/2020

Sky Media to sell BT Sport advertising inventory

BT and Sky have agreed a new multi-year partnership that broadens and deepens their strong commercial relationship. Sky Media, the advertising sales arm of Sky,...

02/07/2020

Brightcove to Power Willie Nelson's Iconic 4th of July Picnic Celebration

Brightcove's live streaming technology continues to revolutionize the event industry through high-quality, immersive live virtual experiences BOSTON, July ...

02/07/2020

Chopin Institute Livestreams Recital Series with Blackmagic Design

Chopin Institute Livestreams Recital Series with Blackmagic Design posted: 02/07/2020 The Fryderyk Chopin Institute's annual summer recital program ha...

02/07/2020

Berlin Brandenburg Airport relies on IHSE KVM solution for control rooms

Berlin Brandenburg Airport relies on IHSE KVM solution for control rooms posted: 02/07/2020 In the new major international airport in Berlin-Brandenburg, KV...

02/07/2020

Video Europe Goes Back to its Roots

Video Europe Goes Back to its Roots posted: 02/07/2020 Video Europe, leading digital cinema, broadcast and edit hire company, has announced an operational...

02/07/2020

MCR and Video Europe Announce Strategic Partnership

MCR and Video Europe Announce Strategic Partnership posted: 02/07/2020 Matt Marner, Steve Green, Mike Ransome MCR Broadcast Hire and Video Europe Ltd tod...

02/07/2020

Enabling secure content collaboration for remote M&E workflows

David Phillips, principal architect, M&E solutions at Cloudian writes that having a secure, cost-effective storage solution that lets employees access tools, ap...

02/07/2020

OTT 2.0: understanding the importance of interactivity and engagement

Ian Sharpe, CEO of Promethean TV, writes that broadcasters can take action to make the most out of the changing landscape to engage customers, and build loyalty...

02/07/2020

Rugby League World Cup 2021, International Rugby League partner on broadcast production

RLWC2021 and the IRL said they will shortly issue an Invitation To Tender for a ...