In vitreous, the collagen fibrils are composed mainly of type II collagen molecules organised around a core of type XI collagen. In the extracellular matrix, collagen molecules assemble to form heterotypic (composed of more than one collagen type) fibrils. Defects affecting this latter protein, therefore, result in an arthropathy with an unaffected or normal ocular phenotype, whereas defects affecting the former will result in ‘full’ Stickler syndrome affecting both ocular and skeletal organisation. While the α1 chain of type XI collagen is expressed in both ocular and cartilaginous tissue, the α2 chain is predominantly expressed in non-ocular (cartilage) tissue. Type XI is expressed in alternative forms in different tissues. Type IX collagen is also a heterotrimer formed by three different constituent chains encoded by three different genes: COL9A1, COL9A2, and COL9A3. In contrast, type XI collagen is formed as a heterotrimer of three different constituent chains each encoded by three different genes: COL11A1, COL11A2, and COL2A1 (the α(1)II peptide interestingly also forms part of the type XI molecule). From this, it will be seen that type II collagen (a homotrimer) only has a single encoding gene, which is called COL2A1 (COL=collagen 2=Type II, A1=α1 peptide) and whose locus is on the long arm of chromosome 12. In contrast, the genes encoding their constituent protein-building blocks are given Arabic numeral nomenclature, written (for human genes) in upper case. This repeating motif is believed to be essential for correct trimerisation of the polypeptide chains during assembly of the triple helical domain and construction of the mature collagen molecule.Ĭonvention dictates that the collagen types are designated by Roman numerals. In either case, the amino-acid sequence in each of the α chains contributing to the triple helix conforms to a highly conserved repeating triplet sequence, whereby glycine, the smallest amino acid, occupies every third position. Alternatively, they may be different (heterotrimers) as in type XI collagen, which has three different constituent peptide chains. These may be identical (homotrimer) as in type II collagen, constructed from three identical cords of type II collagen polypeptide chains. Mutations affecting the genes encoding all three of these fibrillar collagens are now known to cause Stickler syndrome, but the majority are due to defects in the major structural constituent, type II collagen.Īll collagen molecules include a trimer of 3 α peptide chains. The three principal collagen types found in human vitreous are types II, IX, and XI. The collagen family is the most abundant protein group found in animals and consists of a heterogeneous group of extracellular glycoproteins incorporating at least one triple helical domain as their signature motif. The ophthalmologist has a particularly important role in the identification and diagnosis of this latter group. The purpose of this article is, therefore, to bring the ophthalmologist up to date with an overview of the general systemic aspects of the disorder, differential diagnoses, and particularly the novel variations and subgroups with predominantly or exclusively ocular involvement. The last major review was in 1999 3 and research over the last 10 years has brought much new information to light. Initially considered a monogenic disorder (and possibly allelic with Wagner vitreoretinopathy-see below), it has now emerged as the most common manifestation from the spectrum of type II/XI collagenopathies, which also includes spondyloepiphyseal dysplasia congenita (SEDC), Kniest dyplasia, metatropic dysplasia, and achondrogenesis type II. 2 The syndrome is an hereditary connective tissue disorder of fibrillar collagen associated with congenital megalophthalmos, retinal detachment, deafness, cleft palate, Pierre–Robin sequence, joint hypermobility, and premature arthritis. The first report detailing hereditary arthro-ophthalmopathy, or Stickler syndrome as it quickly became known, was in 1965, 1 with a supplementary report 2 years later.
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Lets you make raw music tracks and a few related data, including lyrics, and send them into various distinct formats.Lets you do jobs and also then edit the MIDI files.Equipped with an extremely user-friendly interface.A remarkable digital audio program that will allow you to capture, edit addition to mix your music.Adds explanatory text for certain settings of the configuration section for the MIDI remote mapping wizard.Beneath Are several noticeable features that you’ll encounter after Cubase 5 free download. When double-clicking on a surface element, the MIDI remote mapping wizard now opens.MIDI Controller Surface Editor now supports trigger pads and horizontal faders.You'd better ask real music professionals for a better and unbiased answer. Is it better than ProTools, Nuendo, Fruity Loops, or Adobe Audition? It's impossible for us to say. That would depend on the specific needs of each user and up to which price they're willing to pay because, as you probably already know, audio editing software is very expensive at professional levels. Could we say that it's the best without making a mistake? Definitely, not. Obviously, this isn't the only DAW on the market, although it is one of the most complete and powerful. With regard to VST instrument slots, it comes along with 64 and it's compatible with 256 physical inputs/outputs, as we mentioned a few lines above.Ĭubase or ProTools? Or maybe Nuendo or FL Studio? There are no limitations regarding the number of audio/MIDI tracks or instruments. Pro: the most professional or full version of this software which we have been describing until now.Among its limitations, we have to point out that it's only compatible with 32 physical inputs/outputs, it allows us to record and mix up to 64 audio tracks and 128 MIDI tracks, it supports 32 instrument tracks and has 32 slots for VST instruments. Artist: the intermediate version appropriate for experienced music producers.Among its limitations, we have to point out that it's only compatible with 24 physical inputs/outputs, it allows us to record and mix up to 48 audio tracks and 64 MIDI tracks, it supports 24 instrument tracks and has 16 slots for VST instruments. Elements: the most basic version appropriate for home music producers.But it's also obviously the most expensive application in this range since it costs almost 600 dollars. In this review, we're introducing you to the Pro version and it's, without a doubt, the best of the three as we'll see below. Which Cubase is the best one of them all?Īlthough we hadn't mentioned it until now, you should know that in this family of products we can find 3 different programs, each one of which has slightly different features. Compatibility with external instrumentation such as synthesizers and signal processors.Workflows perfectly integrated into musical articulations.Almost 100 audio effects and musical instruments.Smart compositional tools including the Chord Track, Chord Pads, and the advanced Chord Assistant.MixConsole to create professional mixes.32-bit, floating-point, audio engine with up to 192 kHz, and support for 5.1 surround.Most noteworthy featuresĭownload Cubase for PC to be able to make use of the following features and functions: Record, edit, mix, and produce music just like a professional.Ĭreated back in 1989 by the German company Steinberg (well, that sounds like a good name for a beer if you were to ask me), ever since then its successive versions, with 5, 7, and 9 as some of the most popular, have got better and better until they've released this Cubase Pro 9, a real beast when it comes to professional music editing that also allows us to work on several tracks at the same time, record audio from multiple sources, make the most avant-garde mixes and apply over 80 sounds effects to our songs. They have all decided to download Cubase for PC, a MIDI sequencer with audio editing functions exactly what they need for their daily work. But who uses it? From the most popular artists in the world to musicians, producers, and sound engineers. That's not surprising as it's one of the most complete and powerful DAWs or Digital Audio Workstations on the market with over 30 years of experience in this sector. Whether you're a professional in the world of music or you just like to mix your music from your home computer, you've probably heard of the name Cubase. Produced by: Arthur Sarkissian, Rob Van Norden, Yariv Lerner, Chris Milburn, MosheĮxecutive Producers: Avi Lerner, Trevor Short, Jeffrey Greenstein, Jonathan Yunger, Boazĭavidson, Christa Campbell, Lati Grobman, Luke Daniels, Kyle Stroud, Balan Melarkode, Euzebio Munhoz Junior, Claiton R. Rating: R for strong and bloody violence, language, some sexual references andĬast: Michael Keaton, Maggie Q, and Samuel L. Lionsgate and Millennium Media present, in association with Ingenious Media, a Millennium Media / Fourteen Films / I Road production, in association with Campbell Grobman Films / Ingenious Media. Just weeks after the announcement of the film's release date, Lionsgate has released the trailer for The Protg, an upcoming thriller that pits spy vs. As she becomes entangled with an enigmatic killer (Michael Keaton) whose attraction to her goes way beyond cat and mouse, their confrontation turns deadly and the loose ends of a life spent killing will weave themselves even tighter. But when Moody – the man who was like a father to her and taught her everything she needs to know about trust and survival – is brutally killed, Anna vows revenge. Jackson) and trained in the family business, Anna (Maggie Q) is the world’s most skilled contract killer. Rescued as a child by the legendary assassin Moody (Samuel L. 01:23 Where to Stream: The Protg Powered by Reelgood If I told you there was a new movie coming out about a ruthless assassin, you might tell me you’ve seen that movie before. She encounters another contract killer (Keaton) who stands in her way, but luckily he's got a soft spot for Anne. 1 Released Upcoming Undated films References 'Lionsgate Movies Index'. As of November 2017, Lionsgates films grossed approximately 8.2 billion. Since 1997, about 400 films have been released. Moody's death sends Anne on a revenge mission to find the killers. List of Lionsgate films (20202029) This is a list of films produced and/or distributed by Lionsgate. This is the perfect base model catfishing boat for the beginner tournament fisherman. Rembrandt and Anna, two world premier assassins who share a mysterious past from Vietnam, traverse the. Protege Cat Series All Catfish Boats Boat Overview Based off our best selling Cat boat on the market the ProCat 200, this model was designed for the fisherman who are interested in getting into tournament catfishing. Jackson's Moody after he rescued her as a child. All The Protege trailers, news and movie details. Directed by veteran action filmmaker Martin Campbell (CASINO ROYALE, THE LEGEND OF ZORRO), THE PROTÉGÉ should deliver with Maggie Q in the lead along with Michael Keaton.Īnne (Maggie) is an assassin protégé to Samuel L. Jackson, Michael Keaton and Robert Patrick. Lionsgate is a global content platform whose films, television series, digital products and linear and over-the-top platforms reach next generation audiences around the world. Lionsgate's action thriller THE PROTÉGÉ boasts a great cast with Maggie Q, Samuel L. This monster was having a great day and then this happened. Weaker groups of enemies fled when they saw me, which made me feel powerful and also kept me from stumbling into time-wasting encounters with monsters well below my level. It was also easy to evade foes I didn’t feel like fighting thanks to a visual indicator and alarm that sounded when I got too close. There are hundreds of the suckers, and while some are simple color variations, their abilities change up enough to keep things tense and surprising. The best parts of Dragon Quest are the moments where I’m battling the wonderful beasties. Every enemy is lavishly depicted in the open world, and the Akira Toriyama-designed monsters spring to life with gorgeously fluid animations on my stock PS4. With the exception of a few stints on the high seas, there are no random encounters in Dragon Quest XI. Boss battles get downright tense, and my entire party was wiped out more than once, though never often enough to feel frustrating. The vast menagerie of monsters presents unique challenges in every fight, and I found myself eagerly rushing toward new types of enemies just to see what new twists they threw my way. Combat is snappy, tactically satisfying, and full of surprises. Stabby Stabbyĭragon Quest XI battles prove that turn-based battles can be absolutely nail-biting. I’ve been plugging away in Dragon Quest XI’s sprawling environments for well over 65 hours and I just want to keep going.Ĭheck out a large chunk of the latest Dragon Quest XI footage from last week at Gamescom 2018 above. But that simplicity is a purposefully-crafted facade that melts away after a little time in the immense, complex world. On the surface, Dragon Quest XI seems to be a simple return to the single-player RPG format, after deviating into MMO territory for Dragon Quest X. Dragon Quest XI is a distilled, energetic, mathematically elegant epic that takes what I love most about traditional dungeon crawlers and masterfully iterates on the formula. If you’re thinking about buying colored contact lenses online, you probably already know how important it is to be careful where you buy from.
Step 2.1: Copy the following code into a new text document. Right now the Office is activated successfully. Last 5 characters of installed product key: 6MWKPĬ:\Program Files\Microsoft Office\Office16> Activate Office manually using KMS license LICENSE DESCRIPTION: Office 19, VOLUME_KMSCLIENT channel LICENSE NAME: Office 19, Office19ProPlus2019VL_KMS_Client_AE edition Installed product key detected - attempting to activate the following product: \root\licenses16\proplus2019vl_mak_ae-ul-phn.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /setprt:1688Ĭ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /unpkey:6MWKP >nulĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inpkey:NMMKJ-6RK4F-KMJVX-8D9MJ-6MWKPĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /sethst:e8.us.toĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /act \root\licenses16\proplus2019vl_mak_ae-ul-oob.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_MAK_AE-ul-phn.xrm-ms" \root\licenses16\proplus2019vl_mak_ae-ppd.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_MAK_AE-ul-oob.xrm-ms" \root\licenses16\proplus2019vl_mak_ae-pl.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_MAK_AE-ppd.xrm-ms" \root\licenses16\proplus2019vl_kms_client_ae-ul.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_MAK_AE-pl.xrm-ms" \root\licenses16\proplus2019vl_kms_client_ae-ul-oob.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_KMS_Client_AE-ul.xrm-ms" \root\licenses16\proplus2019vl_kms_client_ae-ppd.xrm-msĬ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_KMS_Client_AE-ul-oob.xrm-ms" Microsoft (R) Windows Script Host Version 5.812Ĭopyright (C) Microsoft Corporation. \root\Licenses16\ProPlus2019VL*.xrm-ms') do cscript ospp.vbs /inslic:".\root\Licenses16\%x"Ĭ:\Program Files\Microsoft Office\Office16>cscript ospp.vbs /inslic:".\root\Licenses16\ProPlus2019VL_KMS_Client_AE-ppd.xrm-ms" The system cannot find the path specified.Ĭ:\Program Files\Microsoft Office\Office16>for /f %x in ('dir /b. C:\Windows\system32>cd /d %ProgramFiles%\Microsoft Office\Office16Ĭ:\Program Files\Microsoft Office\Office16>cd /d %ProgramFiles(x86)%\Microsoft Office\Office16 Here is all the text you will get in the command prompt window. Please make sure your device is online and try the command “act” again until you succeed. If you see the error 0xC004F074, it means that your internet connection is unstable or the server is busy. cscript ospp.vbs /setprt:1688Ĭscript ospp.vbs /inpkey:NMMKJ-6RK4F-KMJVX-8D9MJ-6MWKP Make sure your PC is connected to the internet, then run the following command. Step 1.4: Activate your Office using KMS client key. In it the "pihome" folder will be the folder you've shared. If you select "Network" in windows explorer device "RASPBERRYPI" should be found (it may take a minute or two to appear). If you want to permit read only access change writeable to "No"įinally add the pi user by typing this command and entering the pi's password twice: On my Windows machine, I am able to consistently hit read speeds of 50-60MB/s with a peak of 70MB/s. Search for the workgroup line (normally at the top) and if necessary set it to match your workgroup (if you don't need it set then it can just be left as default, works fine for us not changed)īelow it you'll find the wins support line, if there is a # at the start of the line remove it to stop it being commented out and then ensure it is set to "yes", so you should have:Īdd a section at the bottom which contains the settings for the share:Ĭhange path as required ( "path=/" is fine if you want access to the entire drive). 250 range() function, 206 range statement, 203 Raspberry Pi. I am running a Samba share on the Pi, but I'm getting drastically different speeds based on whether the client is a Windows 10 machine or an Elementary OS (Ubuntu/Debian base) machine. Samba contains the SMB protocol, support for the Windows naming service (WINS), and support for joining Windows workgroups. Samba-common-bin contains a tool that you'll need to register users with Samba. So if I was away from home downloading a file to my phone or computer from my cloud at home, it would take a while because my upload speed uploads at less then. Sudo apt-get install samba samba-common-bin The following instructions will set your RPi to share its files on your local network and be accessible by Windows. Dropbox has recently disabled the API used by pwSafe v1. To keep using Dropbox, you need to upgrade to pwSafe 2, which superseded pwSafe 1 in 2014. Do I need to subscribe to sync or otherwise use pwSafe? #Pwsafe mobile mac sync upgrade No, the subscription is just for pwSafe Cloud. TGWOA 31 - Futari no Meikyuu Oujo V Haha Shimai no Soudatsukon Twin Dungeon-Princesses Vol. Can the twins restore order between the human and fairy tale worlds A Tale of Magic Book 1: The first. CHALLENGE WORDS dungeon magnify festival thunderstorm injury Practice. The Twin Dungeons is a Side Quest in Divinity: Original Sin Enhanced Edition. The Land of Stories Book 6: The epic conclusion. human now I am a girl with a twin brother and we both have cat ears and tail. Although we think pwSafe Cloud adds great value to pwSafe, you don't need to subscribe to it to have a fully-functional password manager, including access to Apple's iCloud Drive for syncing. Unit 1 Week 1 The Twin Club Day 4 Day 5 Get Ready to Read Content Knowledge p. TG GTS, Tiny, Shrinking AR Part 1 of 6: Daniel Savage is a lawyer who is. You'll be missing only Cloud Memory and Cloud Backups. How do I use Safari integration on iOS?īefore using it, you need to enable pwSafe's action extension. To do that, tap the share icon and then select the rightmost ellipsis icon on the black and white icon row. Enable pwSafe in the list which will pop up. Then, whenever you need to autofill a form, create a new password or change an existing one, tap the share icon and then the black and white pwSafe icon. You may move that icon to the left by tapping, holding and then dragging it with your finger. worldwide down professional badoo france com subtitrat review 6 sub fails. As a result, she no longer ruled the Candy Kingdom and the King of Ooo replaced her as the new ruler until a rebellion against King of Ooo allowed her to. You could have backups made by pwSafe itself: Since your safes are files you manage, instead of information stored on some database we operate, you need to recover your file. edmonton zim promotional 2 double and launch photos canadian what network. She rules over the Candy Kingdom, but in the Season 6 episode ' Hot Diggity Doom' (the first part of the season's finale), an election was held and she lost to the King of Ooo by a landslide. If you use pwSafe Cloud, your data might be in the Cloud Backups service. Tap/click the little cloud icon and then the "Cloud Backups" text to list what is stored in your account. If using iOS, try the "Restore Backups" function on the settings menu. I don’t have family or friends or anything down here. I was teaching in the community that I grew up in, so leaving that was hard. It didn’t quite go the way we wanted it to go, so I decided to move to Texas. I decided during the Oklahoma walkouts that if what we were asking for didn’t pass, I would leave. There’s an animosity with respect to teachers that’s a function of sexism that, as I watch it happen more and more, bothers me to my core. People talk about teachers in a way they don’t talk about firefighters and police officers, and they’re all public servants. There’s an element of sexism in the way we talk about teachers. It’s not ‘poor me.’ But I don’t want for young 20-somethings who want to be change agents to look at teaching as something they can’t do. We live in a house in the neighborhood where my students are. I’m not in the poor house like some of my colleagues are. I also have a sponsored podcast and I get speaking fees. I make $76,530, but the cost of living in the Tacoma area is much higher than most. But that’s the bargain that I make because I want to make a difference. Basically, every year that I stay in the classroom, I’m taking somewhere between $20,000 and $60,000 a year and setting it on fire in my front yard. I make sure my daughters go to their doctors and dentist appointments, but myself, if I can put it off, I put it off.Īs somebody who has gone out on the speaking circuit and who has lectured at Harvard, I get job offers on a pretty regular basis, so I know what my market value is, and I know what I get paid for teaching. My oldest daughter was a part of Girl Scouts, but we had to cut that. I can’t ask people every summer for money, for help, because even though they understand, as a mom, I feel defeated having to do that over and over again. My youngest daughter has high-functioning autism, and for three years in a row, she went to a special day camp during the summer. I’m terrified that I won’t have anything to put toward my eldest daughter’s college education. When I pay all my bills, there’s nothing left to put in savings. I’ve been running around with a crack in my windshield for three months because I can’t afford the more than $200 deductible on my car insurance. We just want the appropriate amount that helps us take care of our own bills, and be able to go to the doctor whenever we need to or take care of car maintenance. It’s not about wanting a pay raise or extra income, it’s about wanting a livable wage. I’m a single mom, I make about $69,000 a year, and I still depend on my mom sometimes. I have been teaching for almost 20 years. I think they bring it up to show their support, but ultimately, it comes across as, ‘I can’t believe you’re still a teacher.’ I don’t think it’s ever malicious, but it just kind of eats away at you and you start to question your worth and question whether you should stay in education when you do make so little. It’s also humbling when you’re in a grocery store and people who are well intentioned find out somehow that you’re a teacher and suddenly they start talking about how little you get paid. It’s just really humbling to have to tell one of your best friends that, literally, I just don’t have enough money and I won’t by the time the wedding arrives. I don’t feel like I live a life where I am spending money left and right. He understands, and my friends understand, but it’s embarrassing to pretty much say, ‘I’m working 40 hours, 50 hours, 60 hours a week sometimes, and I still can’t afford to go to a friend’s wedding.’ It’s not anything ostentatious. Even with the pay raise that we’re going to get, I couldn’t swing it financially. I started looking up flights, looking up Airbnbs. My friend is getting married in Salt Lake City next month, and he messaged me a month ago and asked if I would be able to attend the wedding. I can’t think of another job - maybe an ER doctor, I don’t know - where I can get the feeling that I do from teaching. There is not another job where you could have as many highs, as many lows, as much connection, as much challenge as there is in teaching. and I get off at 4 p.m., and usually that’s because I have to go to another job. But we are not paid for the work that we do. We do a really good job educating all kids that come before us. The schools in Kentucky are not failing like our governor wants people to think that they are. Teaching is a science and it is an art, and we work really hard to be able to provide for our students. I wish people understood exactly what it takes to make a classroom work. I was really embarrassed when I first had to start doing that because I think of myself as a professional. I donated plasma twice before my first pay day this year just for gas money. I’ve had to take a sick day before because I didn’t have enough gas to make it to school. Right now, I have a broken tooth that I can’t afford to have fixed. He was eventually linked to more than 20 killings. He is pictured being led to a Thai jail in 1977 Sobhraj developed the name 'The Serpent', of which the Netflix series is named after, from his ability to assume other identities in order to evade justice. When the first few students began falling where they stood, the others became alarmed and called the police. In 1976, Sobhraj attempted to drug a group of 60 French students on holiday in New Delhi in an attempt to rob them of passports and cash by giving them sleeping pills disguised as antibiotics.īut this time it backfired when the poison began working a lot faster than he expected. He later murdered Jean-Luc Solomon by poisoning him. He murdered at least two others in Thailand before fleeing to Kolkata, India, where he killed student Avoni Jacob simply to obtain his passport. While they were staying with him, a visit from Hakim's French girlfriend, Charmayne Carrou threatened to expose him, and so he strangled the pair and burned their bodies. His next victim was Vitali Hakim, whose burnt body was found on the road to the Pattaya resort, followed by fiancées Henk Bintanja and Cornelia Hemker, who had been poisoned by Sobhraj and then nursed back to health. This inspired the killer's nickname 'The Bikini Killer'. Her body was found on a beach in Pattaya, Thailand wearing a bikini. It is believed he committed his first murder in 1975, a 21-year-old woman from Seattle called Teresa Knowlton. While he claimed that murders were often accidental drug overdoses, it was later alleged by investigators that his motive for murder was silencing victims who threatened to expose him.Ĭharles Sobhraj is pictured in June 2014 being escorted to court by Nepalese police He charmed and befriended his victims, many of them starry-eyed Western backpackers on a quest for spirituality, with the help of his besotted lover and accomplice Marie-Andrée Leclerc.īy 1975 the killer was joined by Ajay Chowdhury, a young Indian man who would also help him scam tourists by helping them out of situations he had caused, for example providing shelter to victims he had poisoned. The criminal financed his lifestyle by posing as either a salesman or drug dealer to impress tourists, who he then drugged, robbed and often murdered. the return to his country within 15 days.'Īfter a troubled childhood and several prison terms in France for petty crimes, Sobhraj began travelling the world in the early 1970s and wound up in the Thai capital Bangkok. 'If there is not any other pending cases against him to keep him in the prison, this court orders his release by today and. |
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