Sentences with phrase «of fop»

With that, Tarantino brought out the star - studded cast with whom he'd rehearsed the first draft script for three days: Samuel L. Jackson as Major Marquis Warren, Kurt Russell as the cruel bounty hunter John Ruth, Tim Roth as the English «bit of a fop» Oswaldo Mobray, Amber Tamblyn as Daisy Domergue, Walton Goggins as Chris Mannix, Michael Madsen as John Gage, Bruce Dern as Confederate Gen. Smithers, James Parks as stagecoach driver O.B., Denis Menochet as the Frenchman Bob, Dana Gourrier as Minnie, Zoe Bell as Six Horse Judy and James Remar as Jody.

A recent example is his work in Fibrodysplasia Ossificans Progressiva, where he and his team discovered a novel mechanism that explains important aspects of FOP's pathophysiology and pinpoints a new potential route to therapy.

«It took our team of researchers and collaborators more than 15 years of diligent effort to unravel the genetic cause of FOP that had been shrouded in mystery for so long,» says Fred Kaplan, MD, the Isaac & Rose Nassau Professor of Orthopaedic Molecular Medicine, of the 2006 discovery of the mutation that causes FOP.

This heterotopic bone formation is the most clinically relevant feature of FOP.

The balance of expression of mutant and wild - type ACVR1 protein is likely to affect ligand binding dynamics, receptor oligomerization, internalization, and / or activation of downstream signaling pathways, as well as receptor response to environmental triggers that are clinical features of the natural history of FOP.

By contrast, the R206H ACVR1 mutant only slightly enhances the expression of initial and early chondrogenesis markers (collagen type II and aggrecan), consistent with a milder stimulatory effect of the FOP ACVR1 mutation on chondrocyte differentiation.

The company tested that potential therapy, a type of protein known as a monoclonal antibody, on mice with their own form of FOP and lo and behold, they stopped growing unwelcome new bone.

In 2006, Penn researchers led by Kaplan and Eileen Shore, PhD, the Cali - Weldon Professor of FOP Research and a co-author on the current study, discovered how a mutation in the gene for a BMP receptor called Activin Receptor A type I (ACVR1) occurs in all individuals who have classic FOP.

Scientists from the Center for Research in FOP and Related Disorders at the Perelman School of Medicine at the University of Pennsylvania and colleagues examined the critical role of tissue hypoxia, or oxygen starvation, in the induction and amplification of FOP lesions, also called flare - ups.

For the purpose of FOP drug discovery, scientists have normally focused on molecules associated with BMP signaling.

Instead, we found that palovarotene appears to balance the effects of the FOP mutation, effectively restoring near normal growth.

This complicates study of FOP because the trauma associated with acquiring patient cells could stimulate the irregular bone growth.

In an age of fops and toys, Wanting wisdom, void of right, Who shall nerve heroic boys To hazard all in Freedom's fight, - Break sharply off their jolly games, Forsake their comrades gay, And quit proud homes and youthful dames, For famine, toil, and fray?

Not exact matches

«The FOP is very concerned that the partial or total elimination of SALT deductions will endanger the ability of our state and local government to fund these (law enforcement) agencies,» said the letter, distributed to reporters.

I have felt the freedom now for so long a time that I am sure of my relation toward it; and I could no more harbor any of the thieving and depressing influences that once I nursed as a heritage of humanity than a fop would voluntarily wallow in a filthy gutter.

(The idea of that epicene fop, the Earl of Oxford, taking a break from his falconry and hawking or from his documented European travels to supply two plays a year to this rather disreputable band of low «life artisans is risible.)

In 2014, New Zealand and Australia adopted a new, voluntary, interpretive front - of - pack (FOP) nutrition - labeling scheme entitled the Health Star Rating.

Leading sugar and health expert group Action on Sugar, based at Queen Mary University of London, is calling for colour - coded «traffic light» front of pack (FOP) 1 nutrition labelling to be introduced across all food and drink products after exposing many perceived «healthy» cereal brands who have failed to include the Department of Health endorsed colour - coded labelling -LSB-...]

And FOP plays a lot of those songs that people forgot they loved.

That image of Honeyghan as fop might have amused Bumphus, the 28 -1-1 former WBA junior welterweight champion, had he chanced to see it.

The windswept, bumbling fop that is Boris Johnson (who somehow currently presides as the Mayor of London) has seen fit to approve Tottenham's plans to redevelop White Hart Lane into a 56,000 capacity stadium by turning the current North Stand around and building out into the vacant car - park situated directly behind it.

The FOP filed a complaint with the Illinois Labor Relations Board, accusing the city of unfair labor practices.

The rare, debilitating genetic diseases, fibrodysplasia ossificans progressiva (FOP) and progressive osseous heteroplasia, strike in childhood and lead to years of pain and early death.

«The new FOP model already has shed light on the disease process in FOP by showing that the mutated gene can affect different steps of bone formation,» Hsiao said.

In that disease, fibrodysplasia ossificans progressiva (FOP), a mutation triggers bone growth in muscles, alters skeletal bone formation, and limits motion, breathing, and swallowing, among a host of progressive symptoms.

«These different stages represent potential targets for limiting or stopping the progression of the disease, and may also be useful for blocking abnormal bone formation in other conditions besides FOP.

Independent of these preclinical studies, Clementia Pharmaceuticals is currently conducting phase 2 clinical trials in individuals with FOP, based on the 2011 preclinical results published by Pacifici and Iwamoto.

Researchers at the Center for iPS Cell Research and Application (CiRA), Kyoto University, took advantage of this strategy by reprogramming FOP patient cells and then seeking candidate molecules that could explain how the disease initiates.

The extra bone that appears in FOP flare - ups progresses through a cartilage stage before replacement with mature bone cells, following a sequence of bone formation seen during normal skeletal development.

Another coauthor from Penn Medicine, Frederick S. Kaplan, MD, the Isaac & Rose Nassau Professor of Orthopaedic Molecular Medicine and Chief of the Division of Molecular Orthopaedic Medicine, is a world expert in heterotopic ossification (HO), the abnormal buildup of bone that occurs in FOP.

When someone with FOP is born, we don't have any evidence that there is any of the extraskeletal bone that has started.

When she discovered FOP and the work of Frederick Kaplan, a professor of orthopedic surgery at Penn, she realized she had found a puzzle that was «a disease of misregulated cell differentiation,» she says.

All of these patients are pretty similar in the way their FOP develops — in terms of malformation of the big toe that we see at birth.

FOP is one of the most rare genetic diseases known, occurring in about one in two million people, but spontaneous bone development is relatively common in the broader population.

However, despite the presence of mACVR1 in all FOP patients, individuals with FOP do not form bone continuously but rather episodically during flare - ups, an important clue that suggested that something else fuels the process of lesion formation.

In fibrodysplasia ossificans progressiva (FOP), a mutation triggers bone growth in muscles, which limits motion, breathing, and swallowing, among a host of progressive symptoms.

«Our study provides profound insight into the role of cellular hypoxia in FOP flare - ups and shows that cellular oxygen sensing through HIF - 1α is a critical regulator of the BMP pathway and heterotopic ossification in FOP,» Kaplan said.

Most importantly for individuals with FOP, the study identifies HIF - 1α as a therapeutic target — knowledge that will likely contribute to the development of more effective treatments for FOP and related common disorders of heterotopic ossification.

Although that protein didn't progress to a drug, Economides was struck by a picture he happened upon of Harry Eastlack, a FOP patient who had donated his skeleton, with its many extra layers of bone growth, to the Mütter Museum in Philadelphia, Pennsylvania.

Another clue came from the work of Frederick Kaplan at the University of Pennsylvania, an orthopedic surgeon and FOP pioneer who has been studying the disease for years, and who helped lead the team that found the FOP gene.

In 2006, researchers discovered the genetic defect behind FOP: A mutated version of the gene ACVR1, which in patients produces an overactive form of a cell surface protein called a transmembrane receptor.

In studies of cells, the team was surprised to learn that a family of ligands they had never considered as relevant to FOP, called activins, were switching on the mutant form of ACVR1; activins typically curb signaling by the normal receptor.

Researchers there were studying a protein in animals and realized that it might stop the buildup of bone, which led them to learn about FOP.

The few thousand or so people with FOP worldwide live with grueling uncertainty: Some of their muscles or other soft tissues periodically, and abruptly, transform into new bone that permanently immobilizes parts of their bodies.

«We knew injury was a part of it, but we didn't know what injury did to change» the body so that bone growth resulted, says Paul Yu, who studies FOP at Brigham and Women's Hospital in Boston and is a co-author on the new paper.

Kaplan's research suggested that FOP had an inflammatory component — and, as it happened, one of the activin ligands, called activin A, was involved in inflammation.

Twenty years after he first stumbled on FOP, Economides and his colleagues report today that the gene mutation shared by 97 % of people with the disease can trigger its symptoms in a manner different than had been assumed — through a single molecule not previously eyed as a suspect.

1Department of Orthopaedic Surgery, 2Center for Research in FOP and Related Disorders, and 3Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.

Most notably, the studies are based on transient transfection of wild - type and mutant gene constructs into cultured cells or into zebrafish embryos and are unlikely to reproduce the specific mutant gene dose that is present in heterozygous mutant cells in FOP patients.

FOP is a severely disabling musculoskeletal disease characterized by extensive formation of endochondral bone within soft connective tissues.