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![]() The mutated enzyme protein is synthesized, but is less efficient than the wild type. Another mechanism resulting in excessive adrenal androgen secretion especially in NCAH results from the alteration in enzyme kinetics due to the CYP21A2 missense mutations. In fact, some have an over-responsive glucocorticoid response to ACTH stimulation, possibly reflective of subtle adrenal hyperplasia. For example, patients with NCAH usually have no evidence of ACTH or CRH excess. ![]() Unfortunately, the pathophysiology of NCAH (and CAH) is more complicated than this description would suggest. Individuals with NCAH generally have adequate mineralocorticoid secretion. ![]() The excessive ACTH stimulation also results in fasciculata-reticularis zone hypertrophy, resulting in the adrenal hyperplasia typical of the syndrome, and possibly increased adrenocortical nodularity. Elevated 17-OHP, P4, and androstenedione concentrations are typically found. ![]() With decreased P450c21 activity, conversions of 17-hydroxyprogesterone (17-OHP) to 11-deoxycortisol, and progesterone (P4) to deoxycorticosterone, are impaired. The resulting loss of cortisol negative feedback inhibition leads to increased hypothalamic corticotrophin releasing hormone (CRH) and pituitary adrenocorticotrophic hormone (ACTH) secretion. In broad terms, the virilizing forms (simple virilizing, salt-wasting, and nonclassic) of CAH are characterized by mutations that significantly impair cortisol biosynthesis and lead to the accumulation of steroid intermediates proximal to the deficient enzyme. Nevertheless, utilizing rigid criteria to distinguish among salt wasting, simple virilizing and NCAH can be problematic because impaired 21-hydroxylase function represents a continuum of decreased enzyme activity. Roughly, phenotype correlates with molecular genotype and reflects the residual activity of the milder mutation. One-half to two-thirds of individuals with NCAH carry one allele encoding for a severe defect in enzyme function (which would result in classic CAH if present on both alleles) and an allele encoding a mild defect in enzyme function on the other allele. Novel mutations associated with NCAH include R369W and I230T. Other missense mutations associated with NCAH include P30L, P453S, and R339H. This genetic variant is commonly identified among Eastern Europeans especially those of Ashkenazi Jewish descent. The missense mutation, V281L, accounts for at least one of the CYP21A2 alleles for most patients with NCAH. Individuals with NCAH are generally compound heterozygotes bearing different CYP21A2 mutations on each allele. įunctional analysis of mutations associated with NCAH generally indicates a 50–80% loss of enzymatic (21-hydroxylase) function. Functional studies indicate that these mutations result in 0–5% residual enzymatic function. ![]() The majority of the CYP21A2 mutations reported to date are associated with simple virilizing or salt-wasting classic congenital adrenal hyperplasia (CAH). Nevertheless, approximately 10–12 mutations account for the majority of the affected alleles. Most of the mutations result from recombination between the active gene, CYP21A2, and its highly homologous non-functional pseudogene, CYP21A1P (i.e., gene conversion), which is located in close proximity within the HLA region on chromosome 6p21.3. To date, 127 mutations have been reported in CYP21A2 ( ) these mutations range from complete loss of enzyme function to partial enzyme activity. ![]() ![]() ![]() You will also find different types of Gantt charts and other project planning tools. 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![]() ![]() Serviceberry is adaptable to a variety of soils and moisture conditions and is hardy from USDA Zone 4 through 9.ĭue to its adaptability, downy serviceberry offers many uses in the landscape. However, it is also quite amenable to open, sunny sites in cultivated landscapes. In the wild, downy serviceberry is often found in the understory of the forest or at the edge of the woods in partially shaded conditions. ![]() Even in winter, this tree decorates the landscape with its artistic branching pattern and its smooth gray bark, which is highlighted with vertical, silvery streaks. These fruits were also appreciated by Native Americans and early settlers.ĭowny serviceberry is also much appreciated for its fall foliage, which ranges from yellow-gold to orange-red. The sweet, fleshy fruits, which are about 1/3 inch in diameter, ripen from red to purple in early summer and are a favored food of many birds and mammals. They develop into bright green leaves that are 2 to 4 inches long and are a primary larval food source for several butterfly species. The simple, elliptical, finely serrated leaves unfold with a downy fuzz on their undersurface, giving them a silvery appearance. Though the blooms are at their peak for only a short time, they effectively usher in spring and then yield the show to the unfurling foliage. Butterflies and bees appreciate this bounty of nectar so early in the season. The inch-wide, five-petaled flowers are borne in elegant, loose clusters from the tips of the tree’s twigs. The white flowers appear before the leaves emerge, making the blossoms’ impact all the more dramatic. The Georgia Native Plant Society is pleased to announce that downy serviceberry ( Amelanchier arborea) has been selected as the organization’s 2005 Plant of the Year.ĭowny serviceberry is one of the earliest native trees to bloom in early spring. ![]() ![]() ![]() Maybe instead of using Multi region column, you could do multiple entries for each region it’s available in, ex: US, J, EA, etc and price accordingly- or indicate if these are retail or eBay prices. I agree with Ivan- a lot of Japan versions of these games games are much cheaper than their US counterparts, as well as much cheaper to obtain on Japanese soil vs buying on eBay from the west (often half the prices listed!). Super Potato (1 in Tokyo and 2 in Osaka), Trader, Retro Game Camp, Surugaya Specialty Store, Beep, Friends, multiple Book Offs, Retro TV Game Revival, and A-Too Nipponbashi (which had the lowest prices by far). I just returned from a Japan trip where we visited every game shop in the Akihabara district of Tokyo, as well as Den Den Town in Osaka. Hey there, great list! As a huge fan of the console and shmups, this list is much appreciated! The Nintendo Game Boy Advance Shmups Library.The Sony Playstation 2 (PS2) Shmups Library.The Sony Playstation (PS1) Shmups Library.The Sega Genesis / MegaDrive Shmups Library.Shmups 101: A Beginner’s Guide to 2D Shooters. ![]() I hope you find this useful! Be sure to read the full Sega Saturn Shmup Guide for more info! Galactic Attack / Layer Section (Rayforce) ![]()
![]() ![]() The action is a quintessential SLAPP suit designed to suppress negative consumer journalism. In the motion to dissolve the order, attorney Marc Randazza points out that fashioning a libel lawsuit as a tortious interference lawsuit doesn’t change the ultimate goal of the litigation: to silence criticism. This includes a motion to dissolve the restraining order and a preliminary examination of the powdery substance Purple claims is harmless and that HMR claims could be hazardous to purchasers’ health. That review has since been reinstated and given this header image.Īnd HMR has published a long list of court documents it has filed in this case. Purple Innovations immediately returned to court, demanding it find HMR in contempt of its order, in particular pointing to the poo emoji and HMR’s claims about the unconstitutionality of the order and Purple’s alleged disingenuousness in filing the libel suit. I guess we will change its rating to “□.”ĭo you trust a company that, rather than compete in the marketplace, decides that it will just try and sue negative reviews out of existence? This temporary order commands that we take down all reviews, and even cease rating this company with a rating of “Poor.” Yes, indeed, we are no longer even permitted to rate this company as Poor. It immediately posted an article about the case and offered to comply with the letter of the order, but perhaps not its spirit. Honest Mattress Review didn’t care much for this decision - one it had been given no chance to contest. ![]() It wasn’t even allowed to refer to its previous rating of Purple’s mattress. Rather than give HMR a chance to respond, the judge decided the review site could publish nothing further about Purple or the lawsuit. It also alleged HMR was just a front for site owner Ryan Monahan’s brand management work with Purple’s competitor, Ghostbed. Purple’s lengthy filing contained numerous allegations of harm caused by Honest Mattress Reviews’ extended commentary on the white plastic powder covering every mattress Purple ships. A couple of weeks ago, a federal judge in Utah decided prior restraint was the best way to handle a recently-filed defamation suit against Honest Mattress Reviews by Purple Innovations, makers of the Purple Mattress. ![]() ![]() ![]() If you click a link and purchase an item, I get a small commission at no additional cost to you. On the LRV scale, O absorbs all light (black) and 100 reflects all light (white). RGB represents how much Red, Green, and Blue is in each paint color. Quick Tip: This article includes the RGB and LRV value for each paint color. Please visit your local paint store for paint samples before making your final decision! While I worked hard to capture the swatches’ true color, the swatches below will vary some from the actual paint colors. The goal of this article is to help you narrow down which paint color would work well for your home. Below I share information about each paint color, swatches of each paint color, and the colors in rooms and some on cabinets. I spent hours researching the most popular Sherwin-Williams gray paints and narrowed it down to ten favorites. It can be overwhelming to narrow down! Natural light plays a big role in how a color will look, sheen of the paint, the size of the room, and what furniture you have in the space. The article states that “many products that have low content (emissions during manufacturing) have high VOC emissions during installation and application life.Looking to paint a room, cabinets or doors gray? When it comes to finding the perfect shade there are hundreds of pale grays, dark gray, and darker shades of gray. According to an article by USG (a producer and distributor of construction materials) called the VOC Emissions Certificate of Compliance, the test used to determine paint VOCs (EPA’s Method 24) only applies to the manufacturing process and the paint while it’s in its liquid state and thus has no relationship to the outgassing that occurs while the paint is drying. According to the EPA, VOCs “include a variety of chemicals, some of which may have short- and long-term adverse health effects.” We discovered the reality is that a paint’s “low-” or “zero-” VOC designation actually has nothing to do with indoor air quality. Many are manmade, and they are common in the manufacturing of paint, among other things. VOCs, or “volatile organic compounds,” are materials with a high vapor pressure (they evaporate easily at room temperature) and low water solubility. Mark Savino of Mark’s Painting of Tampa, Florida.Turner has been painting for over 30 years. ![]() Kevin Turner, owner of Kevin Turner Painting in Kansas City.These premium paint color samples allow you to preview the final look of any HGTV Home by Sherwin-Williams paint before committing. Choose from paint color samples in each collection that work together in any combination. Davis has been painting for over 15 years. Bring room-to-room harmony inside your home with designer-inspired HGTV Home by Sherwin-Williams Color Collections. Gary Davis of Davis Custom Painting in the Boston area.Barter has over 15 years of experience as a professional painter. Jack Barter, owner of Jack’s Painting Service in Portland, Maine.Campbell has been in the paint industry for over 15 years. ![]() Joe Campbell of Arizona Painting Company, based in Phoenix and Tucson.DuPont is a third-generation painter with over 10 years of experience. Jeff DuPont, owner of Sound Painting Solutions, LLC in Seattle, Washington.Berry has over 12 years experience and has also produced a series of very informative YouTube videos on painting techniques. Chris Berry of B&K Painting in Boise, Idaho.Your items will be ready for curbside pickup in as little as two hours. Young has been painting for more than 30 years and has been featured on Ask This Old House, sharing his expertise on paint brushes. Buy paint online from home anytime You can place orders for paint, stains and supplies online. Jeff Young, owner of Young Ideas Painting and Design in Concord, Massachusetts.Because it’s a nice thick paint, it isn’t likely to run or splatter, and the end result was an even sheen with hardly any brushstrokes, far better than any low- to mid-level paint we’ve used. As part of this guide, we painted two rooms with it and were surprised at how easy it was to brush and roll. Regal Select is also an easy paint to work with, making it ideal for the non-pro. The one thing we heard over and over from nearly every painter we spoke to was that “you get what you pay for.” At a little over $50 a gallon at the time of writing, Regal Select isn’t cheap, but, like other high-end paints, it applies in fewer coats, looks better, and lasts longer than its cheaper competitors. This is particularly important if you’re looking to match an existing color or to get specific painting advice. Unlike some other popular paints, Regal Select is only available at dedicated paint retailers and smaller hardware stores, where our painters feel more comfortable with the expertise behind the counter. ![]() Our painters like Regal Select for its coverage, durability, cleanability, and color selection. ![]() ![]() roller marks, print line, dimple), minor scratches, slight edge wear, and slight corner wear. 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