Samantha912/NLP_A2_WTH huggingface dataset:
Central core disease (CCD), also known as central core myopathy, is an autosomal dominantly inherited muscle disorder present from birth that negatively affects the skeletal muscles. It was first described by Shy and Magee in 1956. It is characterized by the appearance of the myofibril under the microscope.

Signs and symptoms
The symptoms of CCD are variable, but usually involve hypotonia (decreased muscle tone) at birth, mild delay in child development (highly variable between cases), weakness of the facial muscles, and skeletal malformations such as scoliosis and hip dislocation.

CCD is usually diagnosed in infancy or childhood, but some patients remain asymptomatic until adulthood to middle age. While generally not progressive, there appears to be a growing number of people who do experience a slow clinically significant progression of symptomatology. These cases may be due to the large number of mutations of ryanodine receptor malfunction, and with continued research may be found to be clinical variants.

Pathophysiology

Central core disease is inherited in an autosomal dominant fashion. Most cases have demonstrable mutations in the ryanodine receptor type 1 (RYR1) gene, which are often de novo (newly developed). People with CCD are at increased risk for developing malignant hyperthermia (MH) when receiving general anesthesia.

Diagnosis
The diagnosis is made based on the combination of typical symptoms and the appearance on biopsy (tissue sample) from muscle. The name derives from the typical appearance of the biopsy on light microscopy, where the muscle cells have cores that are devoid of mitochondria and specific enzymes.

Respiratory insufficiency develops in a small proportion of cases. Creatine kinase tend to be normal and electromyography (EMG) shows short duration, short amplitude motor unit action potentials.

Treatment
There is no specific treatment but triggering anesthetics are avoided and relatives are screened for RYR1 mutations as these may make them susceptible to MH.

Some people have done well on Salbutamol (tablets or liquid).

References

External links 

Myoneural junction and neuromuscular diseases
Autosomal dominant disorders
Aratozawa Dam () is a dam in Kurihara, Miyagi Prefecture, Japan, completed in 1998.

References 

Dams in Miyagi Prefecture
Dams completed in 1998
Kurihara, Miyagi
Karabük District (also: Merkez, meaning "central" in Turkish) is a district of the Karabük Province of Turkey. Its seat is the city of Karabük. Its area is 790 km2, and its population is 137,428 (2022).

Composition
There is one municipality in Karabük District:
 Karabük

There are 37 villages in Karabük District:

 Acıöz
 Arıcak
 Aşağıkızılcaören
 Başköy
 Bolkuş
 Bulak
 Bürnük
 Burunsuz
 Cemaller
 Çukurca
 Cumayanı
 Davutlar
 Demirciler
 Düzçam
 Gölören
 Güneşli
 Kadıköy
 Kahyalar
 Kale
 Kamışköy
 Kapaklı
 Karaağaç
 Karaşar
 Kayı
 Mehterler
 Ödemiş
 Ortaca
 Saitler
 Salmanlar
 Şenler
 Sipahiler
 Tandır
 Üçbaş
 Yeşilköy
 Yeşiltepe
 Yukarıkızılcaören
 Zopran

References

Districts of Karabük Province
Encarnação is a novel written by the Brazilian writer José de Alencar. It was first published, posthumously, in 1893.

External links
  Encarnação, the book

1893 Brazilian novels
Novels by José de Alencar
Portuguese-language novels
Scrivner is a surname. Notable people with the surname include:

Errett P. Scrivner (1898–1978), American politician
Lee Scrivner (born 1971), American writer and cultural theorist
Skogbygda may refer to the following locations:

Skogbygda, Akershus, a village in Nes municipality in Akershus county, Norway
Skogbygda, Løten, a village in Løten municipality in Innlandet county, Norway
Skogbygda, Sel, a village in Sel municipality in Innlandet county, Norway
Anne Ethel Beloff-Chain, Lady Chain (26 June 1921 – 2 December 1991) was a British biochemist. She worked at the Istituto Superiore di Sanità (1948–1964), Imperial College London (1964–1986) and the University of Buckingham (1986–1991). Her research focused on carbohydrate metabolism and the hormones involved in diabetes and obesity.

Early life
Anne Ethel Beloff was born in 1921 in Hampstead to Semion (Simon) Beloff (born Semion Rubinowicz) and Maria (Marie) Katzin. Her parents were of Russian–Jewish background, and her siblings included the historian Max Beloff, Baron Beloff, the psychologist John Beloff, the journalist Nora Beloff, and the politician Renee Soskin. Her paternal great-grandmother was Leah Horowitz-Winograd, the sister Eliyahu Shlomo Horowitz-Winograd and a descendant of the Hasidic master, Shmelke Horowitz of Nikolsburg (1726-1778). She earned a degree in chemistry from University College London in 1942 before completing a PhD in the biochemistry of skin burns with Rudolph Peters at the University of Oxford. She visited Harvard Medical School in 1946 to perform research and returned to the UK in 1948. In the same year she married Ernst Boris Chain, a biochemist who had won the Nobel Prize in Physiology or Medicine in 1945, and moved with him to Rome.

Career
Beloff-Chain worked at Italy's Istituto Superiore di Sanità (National Institute of Health) with Chain from 1948 to 1964, and held the position of chief research scientist. Her research focused on carbohydrate metabolism and the mechanism of action of insulin in hormonal control of diabetes and obesity. One of her most significant discoveries was that levels of beta-cell-tropin, an insulin secretagogue hormone, were elevated in the blood of obese individuals.

In 1964, Beloff-Chain and her husband were recruited by Imperial College London and her family returned to London, where she took up a biochemistry teaching post at Imperial College London. She was promoted to professor of biochemistry in 1983, but she decided to leave with her research team in 1985. A £250,000 new laboratory was built and she became a professor at the University of Buckingham in 1986. At Buckingham, she received funding from the Clore Foundation to establish and head the Department of Biochemistry, where she worked until her death.

Death and legacy
She died on 2 December 1991 in Camden Town, in northwest London. She is best remembered for her significant discovery that levels of beta-cell-tropin, an insulin secretagogue hormone, were elevated in the blood of obese individuals.

References

1921 births
1991 deaths
British biochemists
Women biochemists
20th-century British women scientists
People from Hampstead
Alumni of University College London
Alumni of the University of Oxford
Academics of Imperial College London
Academics of the University of Buckingham
British people of Russian-Jewish descent
Anne
British expatriates in Italy
Wives of knights
The Marquardt MA-74 was a ramjet engine built by the Marquardt Corporation for use in supersonic target drones in the 1960s.

History
The MA-74 was developed by Marquardt for use as a powerplant for high-speed target drones; its primary use was in the North American Redhead and Roadrunner. A nacelle-type ramjet, it used a normal shock inlet, and was designed to be mounted on top of the aircraft; fuel was ordinary JP-4.

Variants and applications
MA-74-ZAB
North American MQM-42

Specifications (XPJ40-MD-2)

References

Citations

Bibliography

Pulsejet engines
The Chilean Olympic Committee (COCh) (Comité Olímpico Chile) is a non-profit private organization composed of all Chilean sports federations who join it and accept its governance through statutes and regulations. It was created on 20 June 1934 and recognised the same year by the International Olympic Committee. The Committee is the organization in charge of coordinating Olympic activities in Chile. Before the COCH was formed, this duty was carried out by the “Liga Deportiva de Chile” (Chilean Sports League).
 
The motto of the COCH is the Latin phrase “Citius, Altius, Fortius”, meaning “Faster, Higher, Stronger”

COCH mission
The main goals of the Chilean Olympic Committee, as described in Title I, Article III of its statute are:
 Promote and protect Olympic principles, sports activities and sport education in Chile.
 Promote cultural and academic activities related with the Olympic culture, and in general promote physical activities and sports among Chilean population.
 Promote the respect of the Olympic rules and the Olympic Charter in Chile, and promote the Olympic cultures.
 Promote through the different sport federations the teaching of the different sports disciplines.
 Promote sport science and research related to sports; contribute to training teachers and other professionals related to sports activities.
 Fight all kinds of discrimination and violence in sports activities, as well as drugs and doping.
 Organize the participation of Chile in the Olympic Games, Pan-American Games, ODESUR, Pacific Games, and all other regional and international competitions.
 The exclusive representation of Chile in the International Olympic Committee.	
 Promote sports and physical activities for disabled people in Chile, and coordinate the country’s participation in the Paralympic Games.
 Work to unify the efforts of government and non-governmental organizations to promote physical activity and sports, and remain independent of any political, religious or economic influence.

Sports and activities supported by the Chilean Olympic committee

COCH board of directors 
The board of the Chilean Olympic Committee in 2014 is as follows:
 
 President: Neven Ilic Alvarez
 Vice Chair: Miguel Angel Mujica Brain
 General Secretary: Jaime Agliati Valenzuela
 Treasurer: Veronica Rajii Krebs
 First Director: Carolina Sanz
 Second Director: Patricia Lopez
 Third Director: Rodrigo Moreno
 Fourth Director: Eduardo Valenzuela
 Fifth Director: Luis Alberto Santa Cruz

See also
  Chile at the Olympics
 International Olympic Committee
 Sport in Chile

References

External links
 Official website
 

National Olympic Committees
Chile at the Olympics
Sports governing bodies in Chile
 Sports
{|

{{Infobox ship characteristics
|Hide header=
|Header caption=
|Ship class=*Fishing trawler (1925–39)
Vorpostenboot (1939–40)
|Ship type=
|Ship tonnage=, 
|Ship displacement=
|Ship length=
|Ship beam=
|Ship height=
|Ship draught=
|Ship depth=
|Ship decks=
|Ship deck clearance=
|Ship ramps=
|Ship ice class=
|Ship sail plan=
|Ship power=Triple expansion steam engine, 64nhp
|Ship propulsion=Single screw propeller
|Ship speed=
|Ship capacity=
|Ship crew=
|Ship notes=
}}
|}

V 304 Breslau was a German fishing trawler that was requisitioned in the Second World War by the Kriegsmarine for use as a vorpostenboot. Built in 1925 as Weser, she was renamed Breslau in 1939. She was wrecked at the entrance to the Noordzeekanaal in 1940.

Description
The ship was  long, with a beam of . She had a depth of  and a draught of . She was assessed at , . She was powered by a triple expansion steam engine, which had cylinders of ,  and  diameter by  stroke. The engine was built by Seebeckwerft, Wesermünde, Germany. It was rated at 64nhp. It drove a single screw propeller, and could propel the ship at .

HistoryWeser was built as yard number 462 by Seebeckwerft, Wesermünde, Germanay for the Hochseefischerei Bremerhaven. She was launched in May 1925  and completed in July. The Code Letters QVKM were allocated, as was the fishing boat registration BX 178. On 7 June 1929, she was sold to the Nordsee Deutsche Hochseefischerei Bremen-Cuxhaven AG. Her registration was changed to ON 117. Her port of registry was changed to Nordenham. In 1934, her registration was changed to PG 467 and her Code Letters were changed to DNNS.Weser had been renamed Breslau by February 1939 and her port of registry was changed to Wesermünde. On 30 September, she was requisitioned by the Kriegsmarine for use as a vorpostenboot. She was allocated to 3 Vorpostenflotille as V 304 Breslau''. On 17 September 1940, she was wrecked in a storm at the entrance to the Noordzeekanaal near IJmuiden, North Holland, Netherlands with the loss of four of her crew. The wreck could still be seen in 1969 near the north mole at the entrance to the canal.

References

Sources

1925 ships
Ships built in Bremen (state)
Fishing vessels of Germany
Steamships of Germany
World War II merchant ships of Germany
Auxiliary ships of the Kriegsmarine
Maritime incidents in September 1940
World War II shipwrecks in the North Sea
Randa Lynn Williams (née Duncan) is an American billionaire heir to the Duncan family fortune (through Enterprise Products, which remains under family control). She and her three siblings each hold an 8% stake in the family business.

Biography
Randa Duncan was born to Barbara Ann and Dan Duncan. Her father, Dan Duncan, was the co-founder of Enterprise Products.

She has a JD from the University of Houston and a bachelor's degree from Rice University.

Career
After school, she practiced law with Butler & Binion and Brown, Sims, Wise & White. Williams served as the president and chief executive officer (CEO) of Enterprise Products and vice president at Enterprise Products from 1994 to February 2001.

She inherited $3.1 billion upon the death of her father. Due to a temporary repeal in the estate tax law for the year 2010, Duncan became, along with her siblings, one of the first American billionaires to pay no estate tax since its enactment.

In 2019, she purchased Texas Monthly.

Philanthropy
Williams is a member of the board of trustees for the Houston Zoo, was a member of the board of trustees for the Houston Museum of Natural Science since 1995 and its chairman. She was on the trustee boards for the Manned Space Flight Education Foundation, River Oaks Baptist School and the Houston A+ Challenge.

In 2013, Williams was elected as Enterprise Production Partners' non-executive chairman.

Personal life
She lives in Houston with her husband, and they have one child.

References

1962 births
Date of birth missing (living people)
Businesspeople from Houston
Rice University alumni
American businesspeople in the oil industry
American philanthropists
American billionaires
Female billionaires
Living people
Duncan family
Meir Ben-Shabbat (; born 1966) was Israel's National Security Adviser and Chief of Staff for National Security. He was former head of the Southern Region in the General Security Service. In July 2021, Ben Shabbat announced that he will be stepping down from the National Security Adviser post by late August.

Early life 
Ben-Shabbat was born to Aziza and Makhlouf Ben-Shabbat, religious Jews who immigrated from Safi, Morocco. He is the 12th out of 14 children. He grew up in Dimona and studied at a state religious school and later at the Bnei Akiva Yeshiva Ohel Shlomo in Beer Sheva. He wrote for a local Dimona newspaper and a number of publications in southern Israel. Upon his enlistment in the IDF, he was assigned to the Givati Brigade. As part of his service he received several medals including the Presidential Medal of Excellence.

He holds a bachelor's degree in political science from Bar-Ilan University

In 1989 upon completing his army duty, he joined the Shabak and served in various capacities and command positions. His duties included dealing with Hamas in the Gaza Strip during Operation Cast Lead. During his service he served as head of the cyber security department and worked on the prevention of terrorism.

National Security Advisor 
In 2017, he began serving as National Security Adviser and Deputy Chief of Staff for National Security in the Prime Minister's Office.

As part of his role, he was involved in Israel's fight against COVID-19.

Abraham Accords 

Ben-Shabbat played a role in establishing ties with Israel and the United Arab Emirates, Morocco and Bahrain as part of the US brokered Abraham Accords. In October 2020, he joined US Treasury Secretary Steve Mnuchin and other Israeli and American officials on the first official business delegation to the UAE. He led the Israeli delegation to Bahrain.

References 

1966 births
Living people
People from Dimona
Israeli people of Moroccan-Jewish descent
Tornelasmias capricorni was a species of minute air-breathing land snail, a terrestrial pulmonate gastropod mollusk or micromollusk in the family Achatinellidae. This species was endemic to Blackburn Island, off the coast of Australia; it is now extinct.

References

Capricorni
Extinct gastropods
Taxonomy articles created by Polbot
The Council of Khalistan is a non‐profit political organization based in Washington, D.C., United States. It is seeking to create a homeland for Sikhs by establishing a sovereign state called Khalistan. The Council of Khalistan is one of the key fighting forces of the Khalistan movement. It played important role during the Insurgency in Punjab.

The organization was created On 12 April 1980, when separatist leader Jagjit Singh Chohan officially announced the formation of the Council of Khalistan at Anandpur Sahib and declared himself to be the president. Balbir Singh Sindhu as its Secretary-General. Chohan presented himself as the president of the Republic of Khalistan, set up a cabinet, and issued Khalistani passports, stamps, and currency. On 13 June 1984, Chohan announced a government in exile. On 24 January 1993, It was briefly admitted in the UNPO, as a representative of Khalistan, but was suspended in just a few months.

List of presidents 
The president of the Council of Khalistan is the leader and head of the organization. The title was created in 1980 when Jagjit Singh Chohan declared himself to be the president. There have been three presidents of CoK since the post was established.

See also
Jagjit Singh Chohan
Gurmit Singh Aulakh
Khalistan movement
Khalistan Referendum
Insurgency in Punjab, India

References

External links
Council of Khalistan
 Khalistan.net

Organizations based in Washington, D.C.
Khalistan movement
Sikh politics
History of Punjab, India (1947–present)
Nationalism in India
Arthur Bailey (1903-1979) was a British architect known for his work on churches and their reconstruction after World War II. He formed a partnership with William Henry Ansell.

His work includes:
Restoration of Dutch Church, Austin Friars, London
Restoration of St George's in the East, London
Holy Trinity, Twydall, Medway

References

English architects
1903 births
1979 deaths
Christian Bottollier (25 July 1928 – 1 July 2021) was a French footballer who played as a forward or midfielder.

Biography
Bottollier began his career with , where he played alongside Étienne Mattler. In 1949, he was recruited to play for FC Nancy as a forward. With Nancy, he was a finalist for the Coupe de France in 1953. However, he retired in 1958 following a serious knee injury.

Christian Bottollier died in Saint-Max on 1 July 2021 at the age of 92.

References

1928 births
2021 deaths
French men's footballers
FC Nancy players
Footballers from Marne (department)
Men's association football forwards
Men's association football midfielders
Great Basin National Heritage Area is a federally designated National Heritage Area in Nevada and Utah, including White Pine County, Nevada and Millard County, Utah. The area was designated to recognize and promote the scenic and cultural resources associated with this central portion of the Great Basin.

The National Heritage Area includes Great Basin National Park and portions of Humboldt-Toiyabe and Fishlake National Forests, as well as Fort Deseret, Sevier Lake and the Topaz War Relocation Center.

Great Basin National Heritage Area was designated on October 13, 2006.

The name was changed from Great Basin National Heritage Route to Great Basin National Heritage Area in 2023.

References

External links
 Great Basin National Heritage Area website

 
White Pine County, Nevada
Millard County, Utah
National Heritage Areas of the United States
History of White Pine County, Nevada
2006 establishments in Utah
2006 establishments in Nevada
Protected areas established in 2006
The 1983 British Grand Prix (formally the XXXVI Marlboro British Grand Prix) was a Formula One motor race held at Silverstone on 16 July 1983. It was the ninth race of the 1983 Formula One World Championship.

The 67-lap race was won by Alain Prost, driving a factory Renault, after he started from third position. Nelson Piquet finished second in a Brabham-BMW, with Patrick Tambay third in a Ferrari. The win, Prost's third of the season, enabled him to extend his lead in the Drivers' Championship to six points.

Qualifying

Qualifying report
The front row of the grid was filled by the Ferraris of René Arnoux and Patrick Tambay, Arnoux over 0.6 seconds ahead. Arnoux was the first driver ever to lap Silverstone in under 1:10. Drivers' Championship leader Alain Prost was third in his Renault, with Elio de Angelis in the Lotus alongside him on the second row. The two Brabhams made up the third row with Riccardo Patrese ahead of Nelson Piquet, while on the fourth row were Eddie Cheever in the second Renault and Manfred Winkelhock in the ATS. Completing the top ten were Andrea de Cesaris in the Alfa Romeo and Derek Warwick in the Toleman. Further down the grid, the Spirit team with its Honda turbo engine made an impressive debut as Stefan Johansson qualified 14th, ahead of both McLarens and just one place behind reigning World Champion Keke Rosberg in the Williams.

Qualifying classification

† — Time disallowed.

Race

Race report
At the start of the race, Tambay moved ahead of Arnoux, while de Angelis quickly retired with a turbo failure. Cheever's engine failed on lap 4, while Johansson dropped out on lap 6 with fuel system problems. When Patrese's turbo gave up on lap 10, Piquet moved up to fourth behind the Ferraris and Prost, with de Cesaris fifth and Winkelhock sixth. Prost overtook Arnoux on lap 14, followed by Piquet five laps later. Then, on lap 20, Prost passed Tambay for the lead, with Piquet moving into second on lap 31. In the later stages of the race, Winkelhock's engine failed and de Cesaris dropped behind teammate Mauro Baldi, while Nigel Mansell, who had qualified only 18th in the second Lotus, made a charge through the field, passing Arnoux for fourth on lap 48. At the chequered flag, Prost was 19 seconds ahead of Piquet with Tambay, Mansell, Arnoux and Niki Lauda completing the top six.

In the Drivers' Championship, Prost doubled his lead over Piquet to six points, with Tambay two points further back. Renault moved into the outright lead of the Constructors' Championship, three points ahead of Ferrari.

Race classification

Championship standings after the race

Drivers' Championship standings

Constructors' Championship standings

References

British Grand Prix
British Grand Prix
Grand Prix
British Grand Prix