PAH-specific ACMG/AMP criteria applied: PM2: Absent from 1000G, ESP. gnomAD MAF:0.00004.; PP3: Predicted deleterious in SIFT, Polyphen-2, MutationTaster. REVEL=0.899; PM3_Strong: Detected in trans with V245A and R261X, both pathogenic (PMID:7981714; PMID:16601866); PP4_Moderate: Detected in 3 patients (1 HPA, 1 PKU). BH4 deficiency excluded in 2 patients. (PMID:8533759; PMID:7981714; PMID:9012412; PMID:16601866). In summary this variant meets criteria to be classified as likely pathogenic for phenylketonuria in an autosomal recessive manner based on the ACMG/AMP criteria applied as specified by the PAH Expert Panel: (PM2, PP3, PM3_Strong, PP4_Moderate).
Absent from 1000G, ESP. gnomAD MAF:0.00004.
Detected in 3 patients (1 HPA, 1 PKU). BH4 deficiency excluded in 2 patients.


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N O R T H A M E R I C A N B I R D S 554 milion Flycatchers continue to be spread widely though thinly as breeders in the Region; a con- tinuing pair with chick was at P.M.N.A.S. 25 Jul (FF), and the species nested at Ramona, San Di- ego, with a male and 2 juvs. there 3 Jun (JMcM). Only 2 Brown-crested Flycatchers were at the n. end of their range at S.F.K.R.P. 4 Jun–2 Jul (RAB). An early fall vagrant Eastern Kingbird was at Colorado Lagoon in Long Beach, Los Angeles 6-7 Jul (MSc). An ad. Cassin's Kingbird was feeding a nestling at Deep Springs 15 Jun (SLS); this species breeds only casually in Inyo. A female Scissor-tailed Flycatcher was at Santa Paula, Ventura 17 Jun–22 Aug (GT); mated with a Western Kingbird, it was frst seen on a nest 23 Jun, and at least one young was noted begin- ning 16 Jul. Other Scissor-taileds were at Can- ada Larga, Ventura 5 Jun (TG, IC), P.M.N.A.S. 24 Jun – mid-Jul (MR), Jacumba, San Diego 20 Jun (EGK), and Desert Center, Riverside 10 Jul (CMcG). A Plumbeous Vireo at Santa Rosa Spring in the Santa Rosa Mountains, Riverside 7 Jun (JD, PU) was outside the species' known breeding range. A Warbling Vireo at California City, Kern 13 Jul (TEW) was apparently a very early migrant. Red-eyed Vireos appeared in Los Angeles at Playa Vista 26 Jun (DS), Pasadena 20 Jul (DD), and The Village Green in the Los An- geles Basin 23 Jul–23 Aug (DS); another was at Point Loma, San Diego 4 Jul (KA). After frst appearing last Feb, 3 Clark's Nut- crackers continued through at least 29 Jun in the Laguna Mountains, San Diego (DK). A mag- pie with a black bill on Santa Rosa Island 15-20 Jun (PK) was undoubtedly an escapee, but from where; from vocalizations, it was thought to be Black-billed rather than Eurasian. A Mountain Chickadee at Huntington Beach 12 Jun (BH) was at an unexpected locality in Orange, well away from foothill and s. county areas where this species has bred in the past. A Verdin at Hansen Dam, Los Angeles 23 Jul (KLG) was out of range on the coastal slope, at least 35 km from nearest breeding sites. An early aculeata White-breasted Nuthatch was at G.H.P. 13-27 Jul (TEW). A House Wren at Brawley 27 Jul (HBK) was the earliest ever fall migrant in the Salton Sink. An early migrant Marsh Wren was at G.H.P. 13 Jul (TEW); another at Playa del Rey, Los Ange- les 20 Jul (DS) was within the breeding range of the restricted coastal subspecies clarkae but thought perhaps to be an early fall migrant of another subspecies. White-eyes, provisionally identifed as Japanese White-eyes, have been increasing in numbers in Orange, as illustrated by a count of 10 at Costa Mesa 24 Jul (TFH). A very late spring migrant Swainson's Thrush was at Inyokern, Kern 19 Jun (RAB). A Hermit Thrush on Big Pine Mountain, Santa Barbara 14 Jun (PAGa) was in an area with few sum- away from the breeding areas at S.F.K.R.P. and on the Colorado River near Blythe. Up to 4 White-winged Doves at Ridgecrest, Kern after 1 Apr attempted to nest but failed (RAB); one at Bakersfeld, Kern 25 Jun (JCW) provides one of a very few records for the San Joaquin Valley; and singles at West Los Angeles 20-25 Jul (JE) and at Los Osos, San Luis Obispo 13 Jul (GPS) were the only birds reported along the coast. An Inca Dove at Borrego Springs, San Diego since 15 Dec 2013 fedged 2 young from a nest 14 Jul (RT, THu), but the identity of the other parent remains a mystery. In the high San Gabriel Mountains, Los Ange- les, Flammulated Owls appeared more numer- ous than in past years, as suggested by 11 heard vocalizing during the night of 8-9 May (LAMB). A pair of Elf Owls at a remote desert oases in Riverside fedged 2 young in Jun (EAE), this be- lieved to be the only pair known in California. A Northern Saw-whet Owl photographed in the low desert at Thousand Palms, Riverside 15 Jun (GS) was certainly unexpected. Common Night- hawks appeared more widespread in the higher parts of the San Bernardino Mountains than in recent years; this is the only area away from Inyo in Southern California where the species nests; one over Frazier Mountain, 20 Jun (DPe) provides one of few records in Ventura. A Mexi- can Whip-poor-will was still present at Green Canyon in the San Bernardino Mountains 27 Jul (TAB). The number of Black Swifts at Mon- keyface Falls in the San Bernardino Mountains peaked at 12 on 19 Jun (AH). HUMMINGBIRDS THROUGH WAXWINGS A Blue-throated Hummingbird, the Region's frst, visited a feeder on Palomar Mountain, San Diego long enough to be photographed 13 Jul (JB). Single Allen's Hummingbirds, rarely identifed anywhere away from the coastal low- lands, were near El Centro, Imperial 11-16 Jul (KZK) and at Niland, Imperial 18 Jul (GMcC); in the extreme e. part of the coastal lowlands, this species nested at Loma Linda for the third year, this being the only area where this spe- cies breeds in San Bernardino. Gilded Flickers continue at Cima Dome in the Mojave National Preserve in e. San Bernardino, with a pair at a nest cavity 30 Mar (JoG), 2 together at a differ- ent location 24 May (AEK), along with single birds there 9 (CAM) & 21 Jun (MP), this being the only place in California where this species now occurs. A very early fall migrant Western Wood-Pe- wee was at G.H.P. 27 Jul (TEW), and a Pacif- ic-slope Flycatcher at Brawley, Imperial 26 Jul (TAB) was also quite early. Presumably headed in the opposite direction, a Willow Flycatcher on the Carrizo Plain 16 Jun (PAGa) was the lat- est ever found in spring in San Luis Obispo. Ver- ally early date for this alcid in California waters, followed by 6 off San Diego in Jul (GN, DPo), 9 off Orange after 28 Jun (DD, TAB), 2 off Los Angeles 28 Jul (DD), 3 off Ventura 13 Jul (DPe), and one off Santa Barbara 19 Jul (SL). Four Laughing Gulls were along the coast of San Diego (AS, PAGi, SW) and Orange (BD) in Jul, along with one at Long Beach all summer (AKL); inland, the frst of the post-breeding birds from w. Mexico arrived at the Salton Sink in mid-Jun (CMcG, GMcC), with numbers in- creasing to over 300 by the end of the month. The virtual absence of hatch-year Heermann's Gulls along the coast indicates a near-complete rangewide breeding failure; inland, and away from the Salton Sea, up to 5 ads. were near See- ley (Fig Lagoon) 1-13 Jun (GMcC), and another was much farther inland and e. of the Sierra Nevada on Tinemaha Reservoir 2 Jun (C&RH). An ad. Sooty Tern was photographed at Bolsa Chica, Orange 5 Jul (RS). Two pairs of Least Terns attended nests at S.E.S.S. (Wister Unit) in May but failed to fedge young; however, the presence of an ad. with a recently fedged young at the same location 13 Aug (GMcC) suggests a successful second nesting nearby. Two Least Terns at P.P. 30 Jun (JSF) and two more at the Prado R.P., San Bernardino 23 Jun (HBK) were the only others reported well inland. An Elegant Tern, rare inland, was near Seeley (Fig Lagoon) 25 Jun (CAM). Gull-billed Terns failed to fedge any young at the Salton Sea due to predators and other disturbances, so the only successful nesting in California was by the 20 pairs on San Diego Bay. Black Skimmers also failed to fedge any young at the Salton Sea, and a single bird at Prado R.P. 19 Jul (TGM) was the only one reported well inland away from the Salton Sink. Single Yellow-billed Cuckoos at Santa Paula, Ventura 21 Jul (LSH), Camarillo, Ventura 4 Jul (BS), and at the Whitewater Canyon Preserve, Riverside 8-9 Jul (FS, ML) made the only reports s o u t h e r n c a l i f o r n i a This Craveri's Murrelet photographed of San Diego, San Diego County on 15 July 2014, was one of many present in Southern California waters after 7 June 2014. The dark underwings easily distinguish this bird from the similar Scripps's and Guadalupe Murrelets, and the lack of white around the eye also distinguish it from the latter. Photograph by James R. Morris.


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Since when have we Americans been expected to bow submissively to authority and speak with awe and reverence to those who represent us? – William O. Douglas
Ravine Cliff Autumn wallpaper
September 15, 2010 September 15, 2010 thelonggoodbye
The media has counted Democrats down and out for so long that meme has taken hold. Apparently there is some life left, Small-business tax breaks clear a hurdle in the Senate
After the 61-37 vote on the small business bill yesterday, senators are expected to give it final approval, which requires a simple majority. House Democratic leaders have said they would quickly pass an identical version of the bill for Obama's signature.
"Today's vote brings us one step closer to ending the months-long partisan blockade of a small business jobs bill that was written by both Democrats and Republicans,'' the president said in a statement. "This is a bill that would cut taxes and help provide loans to millions of small business owners who create most of the new jobs in this country.''
The new $30 billion fund would be available to community banks with fewer than $10 billion in assets to encourage lending to small businesses. The bill would combine the fund with about $12 billion in tax breaks aimed at both large and small businesses.
The tax cuts in the bill include breaks for small restaurant owners and retail store owners who remodel or build stores. Also, larger businesses could more quickly recover the costs of capital improvements through depreciation. And the measure would allow small-business owners to deduct costs of health insurance for themselves and their families from self-employment taxes for the 2010 tax year.
Just last week some of my favorite pundits were saying this small business bill has a snow ball's chance. I tended to think they were right. It's nice to be wrong once in a while. Now can Democrats get the message out. To be fair and balanced Republicans – who we know from history can always trust with the nation's finances – have their own plan to cure everything from warts to the deficit – Senate Republicans unveil a plan to make Bush tax cuts permanent
Even as they hammer Democrats for running up record budget deficits, Senate Republicans are rolling out a plan to permanently extend an array of expiring tax breaks that would deprive the Treasury of more than $4 trillion over the next decade, nearly doubling projected deficits over that period unless dramatic spending cuts are made.
The measure, introduced by Senate Minority Leader Mitch McConnell (R-Ky.) this week, would permanently extend the George W. Bush-era income tax cuts that benefit virtually every U.S. taxpayer, rein in the alternative minimum tax and limit the estate tax to estates worth more than $5 million for individuals or $10 million for couples.
Aides to McConnell said they have yet to receive a cost estimate for the measure. But the nonpartisan Congressional Budget Office recently forecast that a similar, slightly more expensive package that includes a full repeal of the estate tax would force the nation to borrow an additional $3.9 trillion over the next decade and increase interest payments on the national debt by $950 billion. That's more than four times the projected deficit impact of President Obama's health-care overhaul and stimulus package combined.
Something else we all know is these facts don't matter. You could yell them in a tea nut's ear while showing them a big screen Powerpoint presentation of the numbers and they would still deny the truth. We're way beyond having any kind of civil national debate. Conservatives have become like a two year old crying and throwing toys, They don't care about reason, they just care about getting what they want. Anything less and they'll throw another temper tantrum.
McConnell's plan is not crazy enough for Kentucky senatorial candidate Rand Paul , Rand Paul doesn't understand how budgets, the Senate, math work
But that's not radical enough for rebel libertarian Rand Paul. Should Kentucky voters send Paul to the Senate, he promises not just to vote against, but to actively filibuster every budget bill that's not balanced.
OK! Good on you, Rand. That's true fiscal conservatism. Of course, that means you'll have to single-handedly close a $1.3 trillion budget gap in one year. So … good luck with that one, I guess. You'll need to get rid of like 10 Departments of Agriculture.
Oh, wait, there's one other very small problem: Senators can't filibuster budgets.
culture, economics, politics, wallpaper
It's Official: The Death of Moderate Conservatism
The Death of Moderate Conservatism
Moderate conservatism – remember Dwight Eisenhower, Nelson Rockefeller and in recent history, Bernie Sanders who left the GOP to become an Independent. They're reminders that moderate conservatism has been on a respirator for years. The far right conservative Redstate makes it official. In a I told you so tone, celebrating the ever so courageous primary victory of the tea party wackadoo Christine O'Donnell's , Don't Repeat the Mistakes of the Past
As a friend of mine in the business of campaigns and elections has said, electing moderates simply to secure a majority for Republicans is a self-defeating proposition. We've seen this play out time and again. Career politicians abhor principle, and adore power and fecklessness. Their presence in Washington provides constant aid and comfort to the Left. They dilute the brand, confuse voters and sell out conservatives just at the moment they are needed most.
It's not about being right rather than winning, it's about the definition of winning in the long term, which cannot be done with elected politicians who don't believe in conservatism.
In the end, it's really that simple.
That might be in the ballpark of touching in its embrace of principles, if modern conservatives hand any principles. The Bush era, in which six of those years conservatives dominated all three branches of government, was extremely far right. They gave as record deficits, a war that was no less than the second greatest act of treason after the Civil War, a crumbling infrastructure, deteriorating education ( especially the sciences and math), special interests buying legislation from the Republican K-Street project and the dismantling of regulations pertaining everything imaginable safeguard from pet food to Wall St grand thrift. Where rational people witnesses the utter failure of a deeply anti-American political movement, conservatives such as Redstate and the tea nuts see they only failed by not being even further to the Right. There is little chance conservative will "dilute" their brand. America knows what it is and will be paying for that brand for decades.
Hey Breitbart, look to your left
During his speech at FreedomWorks' September 12 rally on the National Mall, Andrew Breitbart berated the media for portraying tea party ralliers as "racist, sexist, homophobe," and "Islamophobe," but the crowd he addressed was full of inflammatory signs.
The signage is at the link. Most of them are more about stupidity and fear than anything. What bunker were these people hiding in from 2000 to 2008 when Republicans created an economy that need to be saved.
Racist and pornography provider Tea Party candidate Carl P. Paladino wins GOP gubernatorial nomination in New York
Buffalo multimillionaire and Tea Party candidate Carl P. Paladino defeated former Rep. Rick Lazio to win the Republican gubernatorial nomination in New York last night. The "volatile newcomer," who forwarded racist and pornographic e-mails to friends, has lent a "potentially destabilizing blow to New York Republicans" who have "historically succeeded by choosing moderates."
Sen. Olympia Snowe (R-ME)
"Stung" by Rep. Mike Castle's (R-DE) primary loss, an "exasperated" Sen. Olympia Snowe (R-ME) "sounded off" about the "shrinking wing" of moderate Republicans in an interview yesterday. Allowing that "Congress isn't working right," she argued moderate Republicans "can't be endangered" if the GOP "want[s] to be a majority party" because "ideological purity at 100 percent is a utopian world."
ethics, history, hypocrisy, spin, values
Hillary Clinton is one of the most ethical and most lied about political leaders in America
Trump Lies 158 Times in 7 days
Trump's Tax Secret, He Doesn't Believe in Paying His Share For Our Military, Fire Fighters or Highways
How Trump gave U.S. steel workers the shaft, used Chinese steel
Trump Has Professional Internet Trolls Spreading Lies About Future President Hillary Clinton
The Trump Foundation, Basically A Tool Trump Uses For Stealing Money
There is No Hillary Clinton Email Scandal
Trump Didn't Have To Be Sleazy, Like His Supporters, He Enjoys It
Seniors in Florida and Ohio, GOP platform breaks Trump's 'no cuts' promise on Social Security and Medicare
American Constitution Society
Americans United for Separation of Church and State
Dahlia Lithwick – Slate Legal Analyst
Iraq Veterans Against the War IVAW
News Hounds
No More Mister Nice Blog
People for The American Way
SourceWatch
The Anonymous Liberal
Whiskey Fire
Forest Waterfall wallpaper - What We Have Learned From Romney, Ryan and The Elite
Antique Map of Europe 1595

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Welcome to my homepage. I am a postdoctoral researcher in Telecommunication Engineering / Computer Science at Universidad Rey Juan Carlos (Madrid-Spain). My primary academic interests are graphic processors, image based rendering and computer vision, with applications to consumer electronics and entertainment. Thanks for taking the time to visit.


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