Samantha912/NLP_A2_WTH huggingface dataset:
Boomerang dysplasia is a lethal form of osteochondrodysplasia known for a characteristic congenital feature in which bones of the arms and legs are malformed into the shape of a boomerang. Death usually occurs in early infancy due to complications arising from overwhelming systemic bone malformations.

Osteochondrodysplasias are skeletal disorders that cause malformations of both bone and cartilage.

Presentation
Prenatal and neonatal diagnosis of boomerang dysplasia includes several prominent features found in other osteochondrodysplasias, though the "boomerang" malformation seen in the long bones is the delineating factor.

Featured symptoms of boomerang dysplasia include: dwarfism (a lethal type of infantile dwarfism caused by systemic bone deformities), underossification (lack of bone formation) in the limbs, spine and ilium (pelvis); proliferation of multinucleated giant-cell chondrocytes (cells that produce cartilage and play a role in skeletal development - chondrocytes of this type are rarely found in osteochondrodysplasias), brachydactyly (shortened fingers) and micromelia (undersized, shortened bones).

The characteristic "boomerang" malformation presents intermittently among random absences of long bones throughout the skeleton, in affected individuals. For example, one individual may have an absent radius and fibula, with the "boomerang" formation found in both ulnas and tibias. Another patient may present "boomerang" femora, and an absent tibia.

Cause

Mutations in the Filamin B (FLNB) gene cause boomerang dysplasia. FLNB is a cytoplasmic protein that regulates intracellular communication and signalling by cross-linking the protein actin to allow direct communication between the cell membrane and cytoskeletal network, to control and guide proper skeletal development. Disruptions in this pathway, caused by FLNB mutations, result in the bone and cartilage abnormalities associated with boomerang dysplasia.

Chondrocytes, which also have a role in bone development, are susceptible to these disruptions and either fail to undergo ossification, or ossify incorrectly.

FLNB mutations are involved in a spectrum of lethal bone dysplasias. One such disorder, atelosteogenesis type I, is very similar to boomerang dysplasia, and several symptoms of both often overlap.

Genetics

Early journal reports of boomerang dysplasia suggested X-linked recessive inheritance, based on observation and family history. It was later discovered, however, that the disorder is actually caused by a sporadic genetic mutation fitting an autosomal dominant genetic profile.

Autosomal dominant inheritance indicates that the defective gene responsible for a disorder is located on an autosome, and only one copy of the gene is sufficient to cause the disorder, when inherited from a parent who has the disorder.

Boomerang dysplasia, although an autosomal dominant disorder, is not inherited because those afflicted do not live beyond infancy. They cannot pass the gene to the next generation.

Diagnosis

Treatment

See also
 Larsen syndrome

References

External links 

Congenital disorders
Rare diseases
Autosomal dominant disorders
Mount Warner () is an isolated mountain just south of the head of Arthur Glacier and 5 nautical miles (9 km) north of Mount Crow in the Ford Ranges, Marie Byrd Land. The Spaulding Rocks stand 11 nautical miles (20 km) northeast of the mountain.

Discovered by members of a geological party of the United States Antarctic Service (USAS) (1939–41) and named for Lawrence A. Warner, geologist at the USAS West Base and leader of the party which visited this mountain. 

Mountains of Marie Byrd Land
Treaty of Amity and Commerce may refer to:
 Treaty of Amity and Commerce (United States–France) (1778)
 Treaty of Amity and Commerce (United States–Dutch Republic), a 1782 United States treaty
 Treaty of Amity and Commerce (United States–Sweden) (1783)
 Treaty of Amity and Commerce (Prussia–United States) (1785)
  Treaty of Amity and Commerce (United States-Siam) or Siamese-American Treaty of Amity and Commerce (1833)
  Treaty of Amity and Commerce (U.K.-Siam) or Bowring Treaty  (1855)
  Treaty of Amity and Commerce (U.K.-Japan) or Anglo-Japanese Treaty of Amity and Commerce (1858)
 Treaty of Amity and Commerce (France-Japan) (1858)
 Treaty of Amity and Commerce (United States–Japan) or Harris Treaty (1858)
 Treaty of Amity and Commerce (Prussia–Japan) (1861)
  Treaty of Amity and Commerce (United States-Joseon/Korea) or Joseon-American Treaty of Amity and Commerce (1882)
 Treaty of Amity and Commerce (China-Macau) or Sino-Portuguese Treaty ceding (1887)
  Treaty of Amity and Commerce (United States-Korea) or Korean-American Treaty of Amity and Commerce (1897)

See also
 Jay Treaty or Treaty of Amity, Commerce, and Navigation, Between His Britannic Majesty and the United States of America, a 1794 treaty between the United States and Britain
 Sino-Philippine Treaty of Amity (1947)
 Treaty of Amity and Economic Relations (Thailand–United States)  (1966)
 Treaty of Friendship
Stefan Jarosław Hula Jr. (born 29 September 1986) is a Polish ski jumper, four-time Olympian (2006, 2010, 2018 and 2022), a bronze medalist of the 2018 Olympic Games in team.

Personal life
Stefan Hula Jr. was born in Szczyrk, Poland. His father Stefan Hula Sr. is a former Nordic combined skier and bronze medalist at the 1974 FIS Nordic World Ski Championships in Falun. Hula Jr. has a brother, Przemysław, and two sisters, Katarzyna and Magdalena, who were trained in sledging. In May 2012, he married Marcelina. They run together the company "Huligans" in which Marcelina sews suits for ski jumpers (she sewed the suit, in which Kamil Stoch won two gold medals at Olympic Games in Sochi). Stefan and Marcelina have a daughter Milena (born 2011). In March 2017, he announced the upcoming birth of their second child. On March 23, 2017, his second daughter was born.

Career
At the 2006 Ski Flying World Championships, Hula finished ninth in the team and 37th in the individual events. His best individual World Cup finish was 6th in a large hill event in Kuopio, Finland in 2016. His best individual career finish was second twice in lesser events in 2005. In season 2017/18, he beat his best result and took 5th place in Oberstdorf.

On January 21, 2018 Hula and his teammates Stoch, Kubacki, and Żyła, won the first ever medal, a bronze, for Poland in ski flying in team competition. It was also the first medal for Hula in senior competition. On January 21, 2018 coach Horngacher officially appointed Stefan Hula to 2018 Winter Olympics. On January 27, 2018 he was chosen to team competition in Zakopane. Hula with teammates won team competition in Poland for the first time. It was also a first podium in World Cup for Hula. Next day, in individual competition Hula was leading after 1 round and overall took 4th place, which is his best individual result in World Cup.

Hula made his best at the 2018 Winter Olympics. In normal hill event, he was leading after the first jump, but he ended up in 5th place. On February 19, 2018, Hula and his teammates Maciej Kot, Dawid Kubacki and Kamil Stoch achieved the first medal in an Olympic team competition for Poland. They claimed bronze behind Norway and Germany.

Olympic Games

World Championships

Ski Flying World Championships

World Cup

Season standings

Individual starts

Team victories

References

External links

1986 births
Living people
Polish male ski jumpers
Ski jumpers at the 2006 Winter Olympics
Ski jumpers at the 2010 Winter Olympics
Ski jumpers at the 2018 Winter Olympics
Ski jumpers at the 2022 Winter Olympics
Olympic ski jumpers for Poland
People from Bielsko County
Skiers from Silesian Voivodeship
Olympic bronze medalists for Poland
Olympic medalists in ski jumping
Medalists at the 2018 Winter Olympics
21st-century Polish sportsmen
Mesir Macunu () is a traditional Turkish sweet associated with the city of Manisa. Earlier versions of Mesir macunu were not sweet, but rather spicy in flavor.

Macun is a sweet Turkish confectionery toffee paste that originated from spicy preparations of Mesir macunu.

Herbs and spices used
Below is a list of spices and herbs used in making the Mesir Paste, along with their Turkish and Latin names:

Allspice (Yeni bahar) (Pimenta dioica) 
Alpina officinarum root (Havlıcan kökü) (Alpina officinarium)
Anise (Anason) (Anisum vulgare) 
Black cumin (Çörek otu) (Nigella sativa)
Black Myrobalan (Kara halile) (Terminalia nigra) 
Black pepper (Karabiber) (Piper nigrum) 
Buckthorn (Topalak or Akdiken) (Nerprun alaterne) 
Cardamon (Kakule) (Elettaria cardamomum) 
Cassia (Hiyarsenbe) (Cassia) 
Chebulic myrobalan (Kara halile) (Terminalia chebula) 
China root (Cop-i cini) (Smilax china) 
Cinnamon (Tarçın) (Cinnamomum verum)
Cloves (Karanfil) (Syzygium aromaticum) 
Coconut (Hindistan cevizi) (Cocos nucifera) 
Coriander (Kişniş) (Coriandum sativum)
Cubeb (Kebabe) (Cubebae fructus) 
Cumin (Kimyon) (Cuminum cyminum) 
Dried orange blossom (Portakal çiçeği)
Fennel (Rezene) (Foeniculum vulgare) 
Galingale (Havlıcan) (Alpinia officinarum)
Ginger (Zencefil) (Zingibar officinalis) 
Iksir sugar (Iksir şekeri)
India blossom (Hindistan çiceği)
Java Pepper (Kuyruklu biber) (Piper cubeba) 
Licorice extract (Meyan balı) (Glycyrrhiza uralensis fisch)
Licorice root (Meyan kökü) (Glycyrrhiza glabra)
Mastic (Çam sakızı) (Mastichum)
Millet (Hintdarisi) (Pennisetum glaucum) 
Myrrh (Murrusafi) (Commiphora Molmol) 
Muskroot (Sümbül) (Adoxa moschatellina)
Mustard seed (Hardal tohumu) (Brassica nigra) 
Orange peel (Portakal kabugu) 
Rhubarb (Ravend) (Rheum Palmatum) 
Saffron (Safran) (Crocus Orientalis) 
Citric acid (Limon tuzu) 
Senna (Sinameki) (Cassia senna) 
Turmeric (Zerdeçal) (Curcuma domestica)
Udulkahr (Udulkahir) 
Vanilla (Vanilya) (Vanilla planifolia) 
Woad (Çivit) (Isatis)
Yellow myrobalan (Sarı halile) (Fructus myrobalani)

See also
 List of Turkish desserts

References

Turkish desserts
Turkish inventions
Turkish words and phrases
The Japan national football team in 2014, managed by head coach Alberto Zaccheroni, compete in the 2014 FIFA World Cup, in amongst international friendly matches both at home and abroad, as they progress towards the 2014 FIFA World Cup and 2015 AFC Asian Cup. After the World Cup, Javier Aguirre had been appointed as the new head coach.

Record

Kits

Schedule

Friendly matches

2014 World Cup

Players statistics

Goalscorers

References

External links
Japan Football Association

Japan national football team results
2014 in Japanese football
Japan
The Green Man at Inglewhite is a public house in Inglewhite, Lancashire, England.

The pub, which was established in 1809, has been owned since January 2017 by Mikoh Inns, which also owns The White Bull in Bilsborrow, Lancashire, about three miles to the west.

In 1986, Inglewhite was designated a conservation area. It was appraised again a decade later. A 2011 review "re-evaluates its special architectural and historic interest in line with the requirements of the Town & Country (Listed Buildings and Conservation Areas) Act 1990 using the latest best practice guidance produced by English Heritage." In 2011, Preston City Council designated The Green Man one of eight sites of special interest in the village.

The building is also notable for its use of the now-rare Westmorland slate on its roof.

Gallery

References

External links
Official website of The Green Man at Inglewhite
The Green Man at Inglewhite at Geograph.co.uk

Pubs in Lancashire
Buildings and structures in the City of Preston
1809 establishments in England
Goosnargh
Lewis ab Owen ap Meurig (by 1524 – 1590) of Brondeg, near Newborough, Anglesey, Wales, was a Welsh politician.

Career
He was elected a Member of Parliament (MP) for Anglesey 1553 and 1572 and appointed Sheriff of Anglesey for 1558–1559.

References

|-

Year of birth missing
1590 deaths
Members of the Parliament of England (pre-1707) for constituencies in Wales
16th-century Welsh politicians
People from Anglesey
16th-century births
High Sheriffs of Anglesey
Roncà is a comune (township) in the Province of Verona in the Italian region Veneto, located about  west of Venice and about  east of Verona. As of 1 June 2007, it had a population of 3,585 and an area of .

The municipality of Roncà contains the frazione (subdivision) Terrossa, S. Margherita and Brenton.

Roncà borders the following municipalities: Arzignano, Chiampo, Gambellara, Montebello Vicentino, Montecchia di Crosara, Montorso Vicentino, and San Giovanni Ilarione.

Demographic evolution

Twin towns
Roncà is twinned with:

  Wackernheim, Germany, since 2000

References

See also
Durello (wine)

Cities and towns in Veneto
Michael Hohnstedt (born 3 May 1988) is a German footballer who plays for SpVgg Union Varl.

Career
Hohnstedt ended his professional career at the end of the 2018-19 season and returned home to his former youth club SpVgg Union Varl.

References

External links

Michael Hohnstedt at FuPa

1988 births
Living people
Men's association football fullbacks
German men's footballers
VfB Lübeck players
Sportfreunde Lotte players
VfL Osnabrück players
BSV Schwarz-Weiß Rehden players
3. Liga players
Regionalliga players
People from Ostercappeln
Footballers from Lower Saxony
The Bilingual Research Journal is a triannual peer-reviewed academic journal covering bilingualism. The journal was established in 1975 and is published by Routledge. The current editors-in-chief are Dr. María E. Fránquiz and Emeritus Professor Alba A. Ortiz from the University of Texas at Austin. The Associate Editor is Dr. Gilberto P. Lara from the University of Texas at San Antonio.

See also

National Association for Bilingual Education

External links

Works about bilingualism
Academic journals established in 1975
Multilingual journals
Routledge academic journals
Triannual journals
Linguistics journals
is a fighting game developed by Webfoot Technologies and published by Atari It was released on November 24, 2003 for the Game Boy Advance.

Gameplay
Taiketsu includes 15 characters, including Goku and Broly. Some of those are free to play with instantly, and some can only be obtained by winning fights. The selection of the roster represents a mix of villains and heroes throughout the history of Dragon Ball Z. Each character has his own arsenal of attacks (a projectile and three special attacks), and the power to transform into a more powerful form. All special attacks require a certain part of the super meter, which fills up while standing still and holding down buttons to charge up some energy. Every fight is usually ground-based, though the players can take part in an optional Sky Battle. The one that is on the ground can either accept the challenge or wait around few seconds for the challenger to return.

The game consists of two game modes: Multiplayer and One Player, where One Player consists of events like endurance, sparring, time challenge and tournament and the Multiplayer allows linking with another Game Boy Advance user. There is also a Z-store where the earned in-game currency can be used to unlock music, images and bonus modes and setting.

Reception

Upon its release, Dragon Ball Z: Taiketsu was met with "generally unfavorable" reviews from critics, with an aggregate score of 40% on Metacritic. Hobby Consolas included it as one of the worst video games based on an anime, criticizing the characters art direction. Screen Rant observed that the game felt "unpolished", while giving signs that "no real effort or care went into its creation".

In the United States, the game sold 620,000 copies and earned $14 million by August 2006. During the period between January 2000 and August 2006, it was the 45th highest-selling game launched for the Game Boy Advance, Nintendo DS or PlayStation Portable in that country.

References

2003 video games
Atari games
Taiketsu
Fighting games
Game Boy Advance games
Game Boy Advance-only games
Multiplayer and single-player video games
Video games developed in the United States
Webfoot Technologies games
Mala Mala may refer to:

Mala Mala Airport, airport at Mala Mala, South Africa
Mala Mala Game Reserve, a game reserve located within the Sabi Sand Game Reserve, Mpumalanga province, South Africa
Mala Mala (film), 2014 Puerto Rican documentary film
Ace Young is the self-titled debut album from American Idol 5 contestant Ace Young. The album was released in July 2008 and sold 10,000 copies. The album features Young's single, "Addicted", which landed on number 77 on Billboard's Hot 100 chart.

Track listing
"Addicted" (Andreas Carlsson, Desmond Child, Kalle Engström, Ace Young) – 3:41
"The Letter" (Carlsson, Engström, Young) – 3:33
"A Hard Hand to Hold" (Child, Gary Go) – 3:17
"You Redeem Me" (Diane Warren) – 3:54
"Where Will You Go" (Ness Bautista, Child) – 3:20
"Fast Life" (Julian Bunetta, Sam Horbund, Young) – 3:35
"Young Money" (Bunetta, Young) – 3:47
"How You Gonna Spend Your Life" (Darrell Brown, Child, Kees Dieffenthaller) – 3:47
"The Girl That Got Away" (Carlsson, Child, Engström, Young) – 3:25
"Dirty Mind" (Carlsson, Child, Young) – 3:21
"The Gift" (Child, Engström, Young) – 3:41
"Addicted" (Acoustic) (Hidden) 2:28

Personnel

Maxwell Abrams – tenor sax
David Angell – violin
Chris Baseford – engineer
Ness Bautista – backing vocals, producer
Eric Bazilian – guitar, engineer
Pat Bergeson – harmonica
Bluu Suede – backing vocals
Drew Bollman – assistant engineer
Richard Bravo – percussion, engineer
Julian Bunetta – producer, engineer, mixing
Chuck Butler – acoustic guitar, bass, electric guitar, programming
David Paul Campbell – conductor, string arrangements
Randy Cantor – engineer
Andreas Carlsson – producer
Kirsten Cassell – celli
Will Champlin – backing vocals
Desmond Child – producer, executive producer
Brian Coleman – production coordination
Greg Collins – mixing
Steve Crowder – assistant engineer
David Davidson – violin
Donald Clive Davidson – violin
Sarah Deane – production assistant
Connie Ellisor – violin
Kalle Engstrom – keyboards, programming, producer, engineer, mixing
Marcus Finnie – drums
Ben Fowler – engineer
Gary Go – keyboards, programming, backing vocals, producer, engineer
Jules Gondar – engineer
Carl Gorodetzky – violin, contractor
Ali Helnwein – production coordination
Robert Ikiz – percussion
John 5 – guitar
Chris Kent – bass
Mika Lett – backing vocals
Lee Levin – drums, engineer
Thomas Lindberg – bass
Craig Lozowick – engineer
Gregg Mangiafico – keyboards, string arrangements, string conductor
Dan Muckala – piano, producer, engineer
Justin Neibank – string engineer
John Netti – assistant engineer
Justin Niebank – engineer
Jason Paige – backing vocals
Carole Rabinowitz-Neuen – cello
Lowell Reynolds – assistant engineer
Leslie Richter – assistant engineer
Jay Ruston – engineer
Pamela Sixfin – violin
Mark Smidt – horn
Harry "Slick" Sommerdahl – keyboards
Shane Swayney – guitar, programming, engineer
Will C. Thompson – assistant engineer
Alan Umstead – violin
Catherine Umstead – violin
Mario Valdes – assistant engineer
Gary VanOsdale – viola
Mary Kathryn Vanosdale – violin
Jon Vella – guitar, programming, producer, engineer
Dan Warner – guitar, engineer
Kris Wilkinson – viola
Chris Willis – backing vocals
Ace Young – backing vocals
Neil Zlozower – photography

Charts and sales

References

2008 debut albums
Ace Young albums
In grammar, the prolative case (abbreviated ), also called the vialis case (abbreviated ), prosecutive case (abbreviated ), traversal case, mediative case, or translative case, is a grammatical case of a noun or pronoun that has the basic meaning of "by way of" or "via".

In Finnish, the prolative case follows an established application in a number of fossilized expressions to indicate "by (medium of transaction)". It can be used in other constructions, but then it does not sound 'natural'. Examples would be "postitse" ("by post"), "puhelimitse" ("by telephone"), "meritse" ("by sea"), "netitse" ("over the Internet"). A number of Finnish grammarians classify the prolative form as an adverb because it does not require agreement with adjectives like other Finnish cases. This claim is not true, however, because an adjective will agree with the prolative: "Hän hoiti asian pitkitse kirjeitse" ("He/she dealt with the matter by way of a long letter").

The prolative exists in a similar state in the Estonian language.

The vialis case in Eskimo–Aleut languages has a similar interpretation, used to express movement using a surface or way. For example, in the Greenlandic language  'by ship' or in Central Alaskan Yup'ik  'by river' or  'by sled'.

Basque grammars frequently list the nortzat / nortako case (suffix -tzat or -tako) as "prolative" (prolatiboa). However, the meaning of this case is unrelated to the one just described above for other languages and alternatively has been called "essive / translative", as it means "for [something else], as (being) [something else]"; e.g., hiltzat eman "to give up for dead", lelotzat hartu zuten "they took him for a fool". The meaning "by way of" of the case labelled prolative in the above languages is expressed in Basque by means of the instrumental (suffix -[e]z).

This case is also called the prosecutive case in some languages. It is found under this name in Tundra Nenets, in Old Basque and, with spatial nouns, in Mongolian.

See also
 Perlative case

Further reading

References 

Grammatical cases
Say Nuth Khaw Yum Provincial Park, also known as Indian Arm Park, is a provincial park located in the Lower Mainland of British Columbia, Canada. The park was established on July 13, 1995 by BC Parks to protect the forested mountain terrain of Indian Arm.

History
In 1998, a cooperative park management agreement was signed between BC Parks and the Tsleil-Waututh First Nation.

In February 2010, the park name was changed from Indian Arm Provincial Park to Say Nuth Khaw Yum Provincial Park as part of a second cooperative park management agreement between BC Parks and the Tsleil-Waututh First Nation. Say Nuth Khaw Yum means "Serpent’s Land" in Halkomelem, the traditional language of the Tsleil-Waututh First Nation.

Geography
Say Nuth Khaw Yum Park covers  of mountainous terrain surrounding Indian Arm, an 18 kilometre long fjord that extends north from Burrard Inlet. These lands are part of the traditional territory of the Tsleil-Waututh people who have inhabited the area since time immemorial.

The southeastern corner of the park surrounds most of Buntzen Lake, a small reservoir managed by BC Hydro.

This park also contains a number of significant archaeological sites.

Activities

The park's close proximity to the Greater Vancouver metropolitan area makes it a popular destination for recreation. Popular activities include hiking, camping, kayaking, and scuba diving.

The southeastern corner of the park features a well-developed trail system, most of which are rated as moderately difficult.

See also
Golden Ears Provincial Park

References

External links

Lower Mainland
Provincial parks of British Columbia
1995 establishments in British Columbia
Protected areas established in 1995
Penafiel is a town and a parish in Penafiel Municipality in Portugal. The population in 2011 was 15,552, in an area of 22.52 km². Penafiel has its own station on the Douro railway line.

Industry
AJP Motos, a motorcycle manufacturing company founded in 1987 by António Pinto and Jorge Pinto, is based in Penafiel.

References

Freguesias of Penafiel
Towns in Portugal
Charles Strickland may refer to:
 Sir Charles Strickland, 8th Baronet (1819-1909), barrister and rower
Charles H. Strickland (1916–1988), general superintendent in the Church of the Nazarene
Charles Strickland (town planner) (fl. 1860), local agent and town planner in County Mayo for Lord Dillon
The main character in the 1919 novel by William Somerset Maugham, The Moon and Sixpence

See also
 Charles Stickland (born 1968), senior Royal Marines officer
Galoon is a village in  Gram Panchayat Kadiwan of Rohru tehsil of Shimla district in the Indian state of Himachal Pradesh. It is approximately 48 km from Rohru and 102 km from district headquarters Shimla.

References

 https://schools.org.in/shimla/02112002602/gps-galoon.html

Villages in Shimla district