(2010) identified a heterozygous frameshift mutation in the GPR98 gene ( 602851.0010) and a heterozygous frameshift mutation in the PDZD7 gene ( 612971.0002). In a 51-year-old German man with type II Usher syndrome, who was negative for mutation in the Usher syndrome genes USH2A ( 608400), WHRN ( 607928), and CLRN1 ( 606397), Ebermann et al. Usher Syndrome Type IIC, GPR98/PDZD7 Digenic (2012) concluded that GPR98 mutations account for a small but significant proportion of mutations causing USH2 (6.4%). In the other patient, the PDZD7 gene ( 612971) was analyzed, but no mutations were found. In 2 of the 10 patients, only 1 deleterious mutation was identified in GPR98 screening for large genomic rearrangements revealed a large duplication involving several exons of GPR98 in 1 of the patients. (2012) identified mutations in the GPR98 gene. In 10 of 31 French USH2 patients who were not linked to the USH2A locus ( 608400), Besnard et al. The findings indicated that men can also be affected with USH2C, and that males and females with GPR98 mutations show a typical USH2C phenotype. One German man and his affected sister were compound heterozygous for GPR98 mutations ( 602851.0051.0009). (2009) identified pathogenic mutations in the GPR98 gene in 2 unrelated men with USH2C. (2009) identified a homozygous 136-kb deletion in the GPR98 gene ( 602851.0007) in 6 affected members of an extended consanguineous Iranian family with USH2C, including 3 men and 3 women.Įbermann et al. (2009) concluded that the USH2B locus at chromosome 3p23-p24.2 does not exist and therefore withdrew the locus designation. The family had originally been reported by Hmani et al. (2009) commented that consanguinity can increase familial clustering of multiple hereditary diseases within the same family. Two family members who were doubly heterozygous for both mutations were unaffected at ages 82 and 65 years, respectively. One family member who was doubly homozygous for both mutations had a more severe ocular phenotype. The family also segregated nonsyndromic retinitis pigmentosa-40 (RP40 613801) that was caused by a homozygous mutation in the PDE6B gene ( 180072.0007). Heterozygous mutation carriers were unaffected. In affected members of a large consanguineous Tunisian family with Usher syndrome type II, originally designated as USH2B, Hmani-Aifa et al. (2004) analyzed the GPR98, or VLGR1, gene for mutations in chromosome 5q14-linked Usher syndrome (USH2C) and found mutations limited to 1 isoform, VLGR1b ( 602851.0002- 602851.0005). Usher syndrome, type IIC, GPR98/PDZD7 digenic Usher syndrome, type 2C, GPR98/PDZD7 digenic
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Of course, a few things have been sacrificed to keep the price down, but you will hardly notice that in your coffee brewing experience. Breville BES880BSS Barista Touch Espresso MachineīES880BSS Barista Touch does nearly everything you would expect from a premium Breville machine, but it’s at a lower price point than its rival, the BES 990BSS. Every feature plays a significant role in ensuring your beverage is as great as what you would get from your favorite coffee shop. In the world of semi-automatic espresso machines, the Breville BES870XL Barista Express easily towers over the rest due to its exceptional performance and features. Frequent maintenance is needed to keep it running optimally.Extras and add-ons available to intensify your brewing experience.Superior grinding performance from the conical burr grinder.Clever heating system that produces great taste.Decent control over the coffee brewing experience.It’s therefore not surprising that this model makes one of the best lattes and cappuccinos. You have a manual steam wand as well well known for its signature silky smooth micro-foam milk. What eventually ends up in your cup is a flavorsome, delicious shot. On its part, the machine uses the pre-infusion system to wet the grounds, then activates the nine bars of pressure which results in maximum extraction. With it, it’s up to you to decide the amount of espresso you want in your drink. But if that’s not your cup of tea, use the grind dial. Just learn and master the standard settings for both single and double shots. It may be more hands-on, but the Breville BES870XL won’t disappoint you once you get the hang of it. Be it a built-in grinder, programmable settings, hot water spout, steam wand, you name it, this model has it all. It’s almost unbelievable that you have everything you need here to brew specialty coffees, and yet pay but a mid-range price. Breville BES870XL Barista Express - Best Breville Espresso Machineįor my first Breville espresso machine review, I’m going to talk about the Barista Express, which remains the brand’s most popular model - and for good reason. Breville BES990BSS Oracle Touch Fully Automaticġ. What makes it even more irresistible is that it’s reasonably priced.ĥ. And when it gets down to work, the outcome is no different from what you would get from your favorite coffee shop. It’s super easy to operate, no matter your brewing knowledge or skill. But Breville BES870XL Barista Express isn’t like the rest. You are likely to turn down a semi-automatic espresso machine if user-friendliness is what you are looking for. The goal is to help you find a Breville coffee maker you will be happy to own for the rest of your life. After doing tests, speaking to fellow coffee connoisseurs, and analyzing the feedback on the market, I was able to successfully nail down the best espresso machines from Breville, spread out across different categories. Rather than take a guess and hope that everything turns out right, take a look at this round-up. Maybe you aren’t even sure whether to go for the automatic, super-automatic, or semi-automatic model. Looking at their lineup, I completely understand why one would be spoilt for choice. If you are like me, then you have at one point wanted to own the best Breville espresso machine.īut my guess is you haven’t done that yet. It’s home to some of the greatest coffee machines and kitchen appliances in the market today. Breville may not be a new name if you have been around the coffee industry for a while. Nowadays, you can get the same look while completely eliminating the bulk of the bottom rim. The famous pair worn by Marilyn Monroe in “Gentlemen Prefer Blondes” have nearly clear bottom rims with accented wingtips. While the semi-rimless cat-eye glasses offer a twist on the typical frame, the look itself draws inspiration straight from the 1950s. If that’s the case, take a look at some of these cat-eye styles that are popular spins on the archetypal frame. While vintage cat-eye glasses are still stylish today, you may be looking for something that appeals to more modern sensibilities and embraces the trends of today’s glasses. This Nicole Miller pair combines a semi-rimless frame - a more modern design element - with very vintage black rhinestone cat-eye upper rims. While bejeweled or rhinestone cat-eye glasses are harder to find nowadays, they are still available. Rhinestone cat-eye glassesĬat-eye glasses and rhinestones make a perfect combination and often give cat eyes their elevated, vintage appeal. If you’re interested in a more muted, softer look, gold frames were also popular in '50s cat-eye glasses, but they don’t pop quite as much as black or colored pairs. If you’re looking to capture the look of the cat-eye sunglasses that Holly Golightly made famous, this Hepburn pair will give you the shape. Part of its appeal: You get the playful shape in an understated pattern. The tortoise cat-eye glasses and sunglasses have real staying power, and today, the tortoiseshell look is still popular. Bright pink or purple cat-eye glasses also make a statement similar to red styles but may work better with your skin tone. They offer a modern twist on the classic look but are just as playful and daring. So if you’re looking for red cat-eye glasses, go all out, like with this Moschino pair. The red cat-eye glasses are also a statement-making retro look: These glasses don’t hold back they are striking both in color and design. These Ray-Ban cat-eye sunglasses offer the chunky black look with extended wingtips. While vintage frames may have a few rhinestones or some filigree on the wingtip, you can still find slightly played-down versions. This is the standard look, the kind that conjures up 1950s yearbook photos of young women with chunky black cat-eye glasses. For those interested in the vintage, classic cat-eye glasses look, there are several variations that are retro-chic. Classic cat-eye glassesīecause cat-eye glasses have incorporated so many different trends over their half-century-plus of cultural prominence, you have a lot of options available in cat-eye styles. If you’re looking for the perfect pair of cat-eye glasses, consider updates to some classic styles, or go for a completely modern twist on the iconic look. They draw the eye upward through sophisticated angular lines, complementing face shapes from round to rectangular. These two elements are also what make cat-eye glasses so flattering on so many faces. How are all these different styles considered “cat-eye glasses?” While the exaggerated, upper-rim wingtip at the temple is the telltale sign of cat eyes, the bottom rim that sublimely slopes upward to the top of the wearer’s cheekbone really gives cat-eye glasses frames their signature look. Now you can wear cat-eye glasses in dozens of different ways, from vintage-inspired, brightly colored cat-eye glasses to the subtle eyebrow arch of semi-rimless, wireframe cat-eye glasses. Since then, the eye-catching style has been reimagined over and over again, working its way into nearly every glasses trend across decades. These frames first grabbed our attention as prescription or clear cat-eye glasses in the 1950s, but they really took off when Audrey Hepburn donned her elegant, tortoiseshell sunglasses version in “Breakfast at Tiffany’s.” When you think about vintage glasses, chances are your mind conjures up something in the shape of cat-eye glasses. Her intelligence doesn’t stop there though: Princess Bubblegum can play the trumpet, is fluent in multiple languages and has a good understanding of the disparate cultures that dot the Land of Ooo. Princess Bubblegum is one of the most brilliant people in the world and often creates new inventions that perform impossible tasks. Interests… science, adventures, and the good of her people. In pursuit of this goal, she coordinates the kingdoms defenses, throws lavish parties, and makes regular scientific breakthroughs. Princess Bubblegum runs all the affairs of the Candy Kingdom and has dedicated her life to improving the existence of her subjects. Princess Bubblegum’s primary responsibility as the ruler of the Candy Kingdom is to protect her subjects from the various deadly threats that stalk the land of Ooo. Don’t let the rainbows and whimsy fool you, though: the Land of Ooo can be extremely dangerous. It’s a place full of magic and wonder– while there aren’t many humans around, there’s everything from lava elementals to witches to sentient cookies. In the centuries that have passed since then she’s established herself as the sovereign ruler of the Candy Kingdom, one of the most stable and advanced societies in the Land of Ooo. Princes Bubblegum was born more than 800 years ago out of a sentient mass of bubblegum underground. The Mushroom War irradiated the entire United States, leading to all kinds of unique mutations. Grew up… in the wake of a nuclear war that wiped out almost every human on earth. Her research had led her to believe that outside was not as bad as the silo leaders had told those inside it was. Juliette’s story begins with the description of a cleaning. With the help of Solo, whom she believes is the silo’s only resident, Juliette puts into action a plan to save the man she loves and rescue her people from an evil leader. Her mechanical mindset allows her to survive her own intended death, and she discovers another silo identical to her own. At little Elise’s suggestion, the people decide to head north to water so that they can fish and settle.In Wool Omnibus Edition (Silo Saga 1) by Hugh Howey, new sheriff Juliette Nelson stumbles into a tangle of deceit, manipulation, and murder when she agrees to accept the top law enforcement spot in her apocalyptic underground world. Juliette has realized the truth about Charlotte and her brother. Charlotte comes across them, and is invited by Juliette to join them. Juliette, Jimmy, and the survivors follow the map to a massive concrete bunker stocked with food and supplies – as well as seeds – to begin civilization all over again. They tunnel out from Silo 17 to discover blue skies and green growth, while a computer-controlled veil of dust has been erected around the silos, completing the lie of a destroyed world. Studying a facility map with others, Juliette discovers that each silo has a digging machine directed toward a specific place called “Seed”. Darcy ultimately dies defending Charlotte’s escape, while Keene detonates the bomb and destroys Silo 1. Keene will stay behind to detonate a bomb which will destroy Silo 1, and which will give all other remaining silos a chance to escape. Keene tells Charlotte he is too ill to flee the silo, but that she and Darcy must go instead. Charlotte explains everything to him, during which time he agrees to help her get Keene out of his holding cell. Naturally curious, Darcy thinks there is something big going on. Charlotte is then confronted by a Security agent named Darcy who has figured out that Charlotte had been illegally awakened. Juliette wants nothing to do with Charlotte, believing her to be part of the hierarchy that entrapped humankind in the silos. Juliette blames Keene, unaware of what has happened.Ĭharlotte works to complete the drone, and establishes contact with Juliette. The nearly-5,000 people still in 18 are all killed. Some 200 people manage to escape through the tunnel to Silo 17, with the tunnel being sealed behind them. He gives the extermination order, in which poisonous gas is pumped into the silo. Juliette’s dig succeeds in breaching Silo 17 just as Thurman realizes 18 has gone rogue. He has Keene arrested and brutally beaten. He is both enraged and saddened to learn what Keene has been up to. However, one of the masterminds of the silo project, Senator Paul Thurman, is awakened. Keene races against the clock to prevent this from happening, attempting to talk Juliette through things. The silo with the greatest chance of survival will be released back into the world, while all other silos – including Silo 1 – are exterminated. Intended to save humanity from nanoterrorism, Keene has learned that the fifty silos that have preserved humankind for 250 years so far will be scientifically computed and considered in another 250 years. Keene continues to struggle with the entire concept of the silo system, which he unknowingly helped design. Juliette’s silo contains many residents who are wary about digging through the wall of their silo, but Juliette tells them they have nothing to worry about because she has been to the outside and survived. Meanwhile, Juliette organizes the miners and diggers of Silo 18 to tunnel their way into Silo 17, where Jimmy, a young couple (Rickson and Hannah), and several children, including Elise, wait for contact with 18. Donald Keene’s health has declined dramatically as he tried to find a way to save the other silos, while Charlotte continues work on a modified drone to see what exists beyond the silos. Dust picks up where the events of Shift left off. “Dust” is a dystopian science fiction novel by Hugh Howey, and is the sequel to his novels “Wool” and “Shift”. moddroid promises that any Dungreed mod will not charge players any fees, and it is 100% safe, available, and free to install. moddroid not only provides you with the latest version of Dungreed 1.6.163 for free, but also provides Unlimited Money, Dumb Enemies mod for free, helping you save the repetitive mechanical task in the game, so you can focus on enjoying the joy brought by the game itself. If you want to download this game, as the world's largest mod apk free game download site - moddroid is Your best choice. Train the adventurer and explore deep into the dungeon!Key features:- Procedurally generated, but intricately designed maps- Training system to help explore the dungeon- From rusty swords to cutting-edge sniper rifles, various weapons, and magical items- High-quality appetizing pixel-art of food!- Rebuild the town- Dungeons with varying themes such as a prison, jungle, and lava zone- Exciting monsters, traps and bosses- Controller supportĭungreed As a very popular action game recently, it gained a lot of fans all over the world who love action games. You can permanently increase your stats through training and become even stronger with the assorted items and food found in the dungeon. When an adventurer is slain within the dungeon, his items are lost and he is sent back to town.Don't worry too much though. Enter the dungeon to face the endless threats, rescue the people, and rebuild the town!There are no checkpoints in the dungeon. You must become an adventurer sent from the kingdom to remedy this disaster. Prologue:A quiet and peaceful town was destroyed by a mysterious dungeon and all the townspeople were sucked into it. Dungreed MOD APK 1.6.163, Unlimited Money, Dumb Enemies It will be good in your Freezer for up to 3 months. Simply let if fully cool and individually wrap the different pieces in freezer wrap, a freezer bag, or both. And YES, you can Freeze this Breakfast Casserole. Leftovers can be stored in your refrigerator for 3-4. I’d get it started before bed and put it on low for 6-8 hours and you’ve got breakfast ready the second you wake up! How To Store or Freeze This Recipe You can either cook it on high for 4 hours or low for 8 hours. For the full recipe, just check out our Crockpot Breakfast Casserole Recipe. If you want to wake up to a delicious breakfast aroma you won’t ever forget, you CAN make this casserole in a slow cooker. It reheats great!Īnd, as we talked about above, it’s so easy to tweak and play around with to make just how we want it! Check out the ideas below! Can You Make This In A Crockpot Or Slow Cooker? Just pop it in leftover in the microwave every morning. It’s like a comfort food breakfast combo! And it’s such an easy cleanup! Everything made in just the one skillet and then all in your casserole dish!Īnd, while it’s great to feed a crowd, you can also make it on Sunday and just eat off of it for the rest of the week. Perfect for holiday pitch ins or brunches or church potluck. We love this recipe because it’s great to make and share with a crowd. Then put on more cheese and throw it in the oven. But you really just need to layer sausage, peppers and onions with the tater tots, cheese and egg mixture. Add some diced peppers and onions for some extra flavor and you are in business! AND, you can make this the night ahead if you want, store it in the fridge and throw it in the oven the next morning!įor the full recipe, check out the recipe card below. It’s got all your favorite breakfast flavors in it: Eggs, Sausage, Potatoes (Tater Tots) and Cheese. The brilliance of this dish is it’s simplicity. So, it was only a matter of time before we posted our classic Breakfast Casserole on here! How To Make Tater Tot Breakfast Casserole My wife has been making this Breakfast Casserole (she actually calls it a Christmas Casserole and some people call it a Hotdish) for years! We’ve shared some pretty awesome breakfast casseroles and bakes over the years, like this Blueberry Breakfast Casserole or this Banana Oatmeal Bake. I’m surprised that it’s taken us this long to share this recipe. Need Breakfast Casseroles? You’ve Come To The Right Place! Wake up to a slice of bliss with this delicious Tater Tot Breakfast Casserole! We make ours with Sausage, Eggs, Tater Tots and TONS of Cheese! But there great thing about this recipe is that you can add, subtract and tweak ingredients to your heart’s content! Read below for the recipe and some yummy ideas to make it your own! They record videos that take up much memory on your PC and can’t be loaded quickly to the web. It is not included in Ubuntu repositories and it can be only installed through a PPA.If you need to record a video clip and share it with friends, keep in mind that most screen recorders support only such formats as MP4 or MOV. It's a rather rudimentary screen recorder with limited options.īyzanz records GIF's and runs in a terminal only. Byznaz run on Ubuntu and I didn’t test it on different Linux distributions. One possible solution is byzanz if you don’t mind using the CLI. It can also be run from a terminal as silentcast. You can then remove the PPAs with sudo add-apt-repository -r like so: sudo add-apt-repository -r ppa:sethj/silentcast & sudo apt-get updateįind Silentcast in the menu under either Graphics or Multimedia, type silentcast into the search box, or ALT+ F2 silentcast. Or run the following, condensed, command: sudo add-apt-repository ppa:sethj/silentcast & sudo apt-get update & sudo apt-get install silentcast Uninstall with sudo pacman -R silentcastįor 14.04 and 12.04 run the following commands to install Silentcast (for older versions of Ubuntu follow the "Any Linux Distro" instructions below): sudo add-apt-repository ppa:sethj/silentcast This will do exactly the same thing as an AUR helper would do for installation, but you will have to keep track of updates yourself.
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Stir in around ⅔ of the cheese, reserving some to top. Simmer for about 5 minutes, don’t allow to boil. We don't prefer it on ours, but do what you like!Īdd in the heavy whipping cream very slowly, while whisking continuously. Optional add-ins: If you like breadcrumbs on traditional mac and cheese, substitute them with pork rind crumbs or low carb bread crumbs.We use a mixture of Colby jack, Havarti, mozzarella, cheddar cheese, pepper jack, white cheddar cheese and parmesan cheese. The other "rule" we'd say to follow is that most of the cheeses should have a bit of a milder taste, with a couple sharper type cheeses that give a little bite to balance out the flavors. Cheeses - This southern baked style mac and cheese sauce consists of 7 different cheeses! You can use your favorite varieties, but we do recommend shredding the cheese fresh so it melts properly.It helps thicken the cheese sauce without the use of a roux, or many keto versions that use cream cheese. Heavy whipping cream - This recipe uses heavy cream instead of evaporated milk and whole milk that are used in our traditional recipe.We use: onion powder, garlic powder, our homemade cajun seasoning blend (we will post the recipe below), mustard powder, cayenne pepper, smoked paprika, and of course a little kosher salt and pepper. Keep this in mind and season to yours taste to suit your own preferences. Our family is always so pleased with the taste. Seasoning blend- As we mentioned, we make our mac and cheese slightly spicy, and the kick of heat in this is just right.Unsalted butter - This is the base of the cheese sauce.We always have Great Low Carb Company noodles in our pantry, and while they aren't a super clean gluten free ingredient, we absolutely adore them when the pasta craving strikes. Low carb macaroni noodles - You can use anything you like here, even cauliflower florets or rice if you prefer.Here is a list of ingredients, you can see the recipe card for quantities and nutritional information. Crowd pleaser - We have yet to meet anyone who doesn't like this recipe! All of our friends request it for parties and Chamere's grandma approves.Great for many occasions - Whether you have a special occasion like a holiday get together or you just want a delectable side tonight, this recipe is always in your toolkit.Easy to customize - With this recipe, it's easy to switch out the noodles and cheese for something more tailored to you!.Ultimate comfort food - This mac and cheese is the perfect ooey gooey crispy and cheesy bite!.Keep reading from a note from Chamere, too! He is always behind the camera but wanted to pop in to talk about his love of the recipe. If you are looking for more hearty sides, be sure to check out these air fryer fingerling potatoes, our air fryer spaghetti squash with cheesy garlic filling recipe, and this spinach artichoke dip! It can be used with any noodles you like, but this recipe is actually made without a roux! Our low carb version of this traditional dish is only 6 net carbs per serving! You won't be disappointed. Learn how we take our carb laden version of this family recipe and flipped it. Over the years, we've worked together to create a stellar mac and cheese recipe that is requested by family members, even grandma! This cheesy baked dish was a staple growing up in Chamere's household. So many low carb macaroni cheese recipes are actually cauliflower mac, (like this low carb cauliflower version we have) but we wanted the real thing with low carb noodles. |
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