Adrenoleukodystrophy is a disease linked to the X chromosome. It is a result of fatty acid buildup caused by the relevant enzymes not functioning properly, which causes damage to the myelin sheath of the nerves, resulting in seizures and hyperactivity. Other symptoms include problems with speaking and understanding verbal instructions. In more detail, it is a disorder of peroxisomal fatty acid beta oxidation which results in the accumulation of long chain fatty acids in tissues throughout the body; the most affected tissues are the myelin in the central nervous system, the adrenal cortex, the Leydig cells in the testes. Clinically, ALD is a heterogeneous disorder, presenting with several distinct phenotypes, no clear pattern of genotype-phenotype correlation; as an X-linked disorder, ALD presents most in males, however 50% of heterozygote females show some symptoms in life. Two-thirds of ALD patients will present with the childhood cerebral form of the disease, the most severe form, it is characterized by normal development in early childhood, followed by rapid degeneration to a vegetative state.
The other forms of ALD vary in terms of onset and clinical severity, ranging from adrenal insufficiency to progressive paraparesis in early adulthood. ALD is caused by mutations in ABCD1, a gene located on the X chromosome that codes for ALD, a peroxisomal membrane transporter protein; the exact mechanism of the pathogenesis of the various forms of ALD is not known. Biochemically, individuals with ALD show high levels of unbranched, saturated long chain fatty acids cerotic acid; the level of cerotic acid in plasma does not correlate with clinical presentation. Treatment options for ALD are limited. Dietary treatment is with Lorenzo's oil. For the childhood cerebral form, stem cell transplant and gene therapy are options if the disease is detected early in the clinical course. Adrenal insufficiency in ALD patients can be treated. ALD is the most common peroxisomal inborn error of metabolism, with an incidence estimated between 1:18,000 and 1:50,000, it does not have a higher incidence in any specific ethnic groups.
ALD can present in different ways. The different presentations are complicated by the pattern of X-linked recessive inheritance. There have been seven phenotypes described in five in females. Initial symptoms in boys affected with the childhood cerebral form of ALD include emotional instability and disruptive behavior at school. Older patients affected with the cerebral form will present with similar symptoms. Untreated, cerebral ALD is characterized by progressive demyelination leading to a vegetative state and death. Adult males with an adrenomyeloneuropathy presentation present with muscle stiffness and sexual dysfunction. All patients with clinically recognized ALD phenotypes are at risk for adrenal insufficiency. There is no reliable way to predict which form of the disease an affected individual will develop, with multiple phenotypes being demonstrated within families. Onset of adrenal insufficiency is the first symptom, appearing as early as two years of age. ALD is caused by mutations in ABCD1, located at Xq28 and demonstrates X-linked recessive inheritance.
The gene ABCD1 encodes a peroxisomal membrane transporter, responsible for transporting long chain fatty acid substrate into the peroxisomes for degradation. Mutations in this gene that interfere with this process cause this syndrome. Males with an ABCD1 mutation are hemizygous. Female carriers will avoid the most severe manifestations of the disease, but become symptomatic in life. Although, the detection of an ABCD1 mutation identifies an individual, affected with a form of ALD, there is no genotype - phenotype correlation. Within a family, there will be several different phenotypes, despite the presence of the same causative mutation. In one case, a family with six affected members displayed five different phenotypes. There are no common mutations that cause ALD, most are familial. 600 different mutations have been identified half are missense mutations, one quarter are frameshifts, with in-frame deletions and splicing defects making up the remainder. The incidence of new mutations in ALD is estimated at 4.1%, with the possibility that these are due to germline mosaicism.
The exact cause for the varied collection of symptoms found in the different ALD phenotypes is not clear. The white matter of the brain, the Leydig cells of the testes and the adrenal cortex are the most affected systems; the excess VLCFA can be detected in all tissues of the body, despite the localization of symptoms. The lack of Coenzyme A does not permit the disintegration of the VLCFA, accumulating the same in the white matter, adrenal glands, the testes more in the Leydig cells not allowing the proper function of this organs. Successful treatment of the demyelination process that affects the brain with either stem cell transplant or gene therapy does not normalize the VLCFA levels in body tissues; the levels of VLCFA can be normalized by treatment with Lorenzo's oil, but this does not alter the progression of the disease. It is unclear whether the accumulation of VLCFA is associated with the pathogenesis of the disease in a specific way, or if it is a biochemical phenotype, useful for identification.
The clinical presentation of ALD can vary making d
A Tiene, sometimes called Tine or Obsttiene, was a special container for transporting wine and fruit. These were used until shortly after the First World War in the Brandenburg city of Werder in northern Germany; the wooden tubs were carried on people's backs to small boats and shipped on the Havel River to market stalls in Berlin. The fruit-growing area around the river island city of Werder has a long tradition, back to the fruit fields of the Cistercian monks at Lehnin Abbey, one of the oldest monastic foundations in Brandenberg, established in 1180; the abbey lands produced large amounts of grapes and other fruits which were processed and transported to Berlin. The term Tiene comes from the wooden vats in which the grapes were pressed for processing into wine, used in the 16th and 17th centuries. In the 19th century, fruit growers transferred the term to the small wooden tubs in which they transported their fruit. A Tiene holds 7 litres, or 3.5 to 4 kg mass, depending on the kind of fruit.
The oak tubs weigh 1.8 kg. The conical Tienen were prepared for shipping by covering the top with linen cloth. By about 1900 more than 200,000 Tienen were in use in the Werder region and their production was a significant craft industry in the town. Three coopers worked on the island and it is to be supposed that the cooper's trade began in the 18th century in Werder. Before the Tienen were sold to the fruit farmers, the coopers had to calibrate them; the Tienen were dried and weighed and the dead weight was branded on the outside. A new Tiene for cherries with a capacity of 7 to 9 pounds cost sixty pfennigs in 1908. A Tiene for raspberries weighed 50 to 60 pounds. Werder pioneered these transport containers and when it introduced the'chip' basket in 1910, the Tienen lost their former importance. In his travelogue Wanderungen durch die Mark Brandenburg, Theodor Fontane recalled walking to school in Berlin past the Werder people at their original position for selling their fruit, at Burgstraße between the Friedrichsbrücke and Herkulesbrücke bridges.
At times it happened that from the Unterbaum we saw the late'second wave' of the Werder folks arrive, big barges crammed with Tienen, while twenty Werder women sat on the oar benches, moving with equal energy their oars and their heads in their pannier hats... The air swam within a refreshing aroma, the cupola of inverted and heaped up wooden Tienen was more interesting to us than the commode-shaped Monbijou Palace and, sad to say more interesting than the forest of columns of Schinkel's New Museum. Dr. H.-Joachim Koch. "Blütenstadt Werder/Havel" Heimatgeschichtliche Beiträge 1984 pp. 18–21
Klete D. Keller is an American former competition swimmer who won medals at the 2000 Summer Olympics and the 2004 Summer Olympics in the 400-meter freestyle and the 4×200-meter freestyle relay. In the 4×200-meter freestyle relay, Keller held off a charging Ian Thorpe in the anchor leg to win the race by 0.13 seconds. This was the first time; the American relay of Michael Phelps, Ryan Lochte, Peter Vanderkaay, Keller are undefeated since the Athens games. Vanderkaay, Larsen Jensen, Erik Vendt, Keller make up the core of the premier American mid-distance/distance freestyle swimmers. Klete was one of many 2000 Olympic medalists from The Race Club World Team, a summer swimming camp in Florida. Keller attended the University of Southern California for two years from 2000 to 2001, but left school to focus on swimming. While at USC, Keller won multiple individual and relay Pac-10 and NCAA Championships in the 200, 500 and 1,650-yard freestyle, as well as freestyle relays. Keller has won many U. S. national titles and FINA world championship medals in 200 and 400-meter freestyles and the 4×200-meter free relay.
From 2004 to 2007, Keller trained at the University of Michigan in Ann Arbor under Bob Bowman and former head coach Jon Urbanchek. He has returned to USC to finish school and train under coach Dave Salo. List of Olympic medalists in swimming List of University of Southern California people List of World Aquatics Championships medalists in swimming World record progression 4 × 200 metres freestyle relay Klete Keller at the United States Olympic & Paralympic Committee
"Beat Control" is a digital EP by indie pop group Tilly and the Wall, the name of its title track. The EP was released via Team Love Records to online music stores on March 4, 2008. A limited edition 7" vinyl was released. "Beat Control" is not on the band's third album o. The EP debuted on the UK Singles Chart at #153; the music video for "Beat Control" premiered on February 25. The band perform in fluorescent clothing with a neon glow surrounding them, against patterned backgrounds. Digital EP:"Beat Control" – 2:53 "Cacophony" – 2:27 "L3t Teh B34t C0ns013 Yov" – 3:247" Vinyl:"Beat Control" – 2:53 "L3t Teh B34t C0ns013 Yov" – 3:24CD single:"Beat Control" – 2:47 "Too Excited" – 3:18 Tilly and the Wall official website Tilly and the Wall on MySpace Team Love Records
Legio septima Claudia was a legion of the Imperial Roman army. They were ordered to Cisalpine Gaul around 58 BC by Julius Caesar, marched with him throughout the entire Gallic Wars; the Roman commander mentions the Seventh in his account of the battle against the Nervians, it seems that it was employed during the expedition through western Gaul led by Caesar's deputy Crassus. In 56 BC, the Seventh was present during the Venetic campaign. During the crisis caused by Vercingetorix, it fought in the neighborhood of Lutetia. Legio VII was one of the two legions used in Caesar's invasions of Britain, played a crucial role in the Battle of Pharsalus in 48 BC, it existed at least until the end of the 4th century, guarding the middle Danube. Tiberius Claudius Maximus, the Roman soldier who brought the head of Decebalus to the emperor Trajan, was serving in Legio VII Claudia. An inscription in Pompeii revealed that a certain Floronius served in the seventh legion; the inscription says: "privileged soldier of the 7th legion, was here.
The women did not know of his presence. Only six women came to know, too few for such a stallion." List of Roman legions livius.org account D. Toncinić, Monuments of Legio VII in the Roman Province of Dalmatia. Split, 2011
Leif Gustav Willy Persson is a Swedish criminologist and novelist. Persson has four children, one of whom, Malin Persson Giolito, is a crime writer. Leif Gustav Willy Persson was born on 12 March 1945 in Stockholm, Sweden to Gustav and Margit Persson. In 1977, while working at the Swedish National Police Board, Persson was the whistleblower who worked with journalist Peter Bratt in the so-called Geijer Scandal when he confirmed a classified memo sent by National Police Commissioner Carl Persson to Prime Minister Olof Palme about the alleged ties of the Minister of Justice, Lennart Geijer, to a prostitution ring in Stockholm. Following this affair he was fired from the National Police Board; the string of events drove Persson to suicide, but he soon returned as lecturer at Stockholm University. The prostitution ring affair inspired him to write Grisfesten, he returned to the National Police Board as a professor in 1992. Persson was a professor in criminology at the Swedish National Police Board from 1992 to 2012.
He is well known in Sweden for his crime fiction novels and for his regular appearances as an expert commentator on notable crime cases on television and in newspapers. Between 1999 and 2009, he participated as an expert commentator on the TV3 television show Efterlyst, about wanted Swedish criminals. Between 2010 and 2018, he participated in the SVT television show Veckans Brott with Camilla Kvartoft, about unsolved Swedish criminal cases both recent and cold. Grisfesten Profitörerna Hidden criminality: theoretical and methodological problems, empirical results Min kompetens Samhällsbärarna Mellan sommarens längtan och vinterns köld. En annan tid, ett annat liv Linda - som i Lindamordet Faller fritt som i en dröm Den som dödar draken Den döende detektiven Gustavs Grabb' Den sanna historien om Pinocchios näsa Bombmakaren och hans kvinna So far, three of Persson's novels feature homicide detective Evert Bäckström. Bäckström is rude, overweight, racist and cynical. In spite of this, he displays excellent deductive skills.
Bäckström appeared as a supporting character in the TV mini-series En pilgrims död and Den fjärde mannen. In both of these series, Bäckström was played by Claes Malmberg. An American TV Series was made in 2015 entitled Backstrom; the show takes place in Portland, Oregon, USA instead of Sweden, the character's first name is changed to Everett. However the character's actual personality remains less unchanged. Backstrom was played by Rainn Wilson; the show was cancelled after one season. Several of Persson's novels feature police officers Bo Jarnebring and Lars Martin Johansson and best friends. In the film Mannen från Mallorca Jarnebring is played by Sven Wollter, Johansson by Tomas von Brömssen. Two TV mini-series featuring the characters were produced starring Rolf Lassgård as Johansson and Per Svensson as Jarnebring: En pilgrims död and Den fjärde mannen. Created by Persson, police detective Anna Holt has appeared in both Persson's novels, in novels by fellow author Jan Guillou. Holt has appeared in several television adaptations starring Petra Nielsen.
Profitörerna Mannen från Mallorca I lagens namn En pilgrims död Den fjärde mannen Backstrom, Persson won the Best Swedish Crime Novel Award, for three of his novels: Samhällsbärarna in 1982, En annan tid, ett annat liv in 2003, Den döende detektiven in 2010. This award is a literary prize awarded annually by the Swedish Crime Writers' Academy, he received the 2011 Glass Key award for Den döende detektiven. Leif G. W. Persson on IMDb Leif G. W. Persson's blog The Salomonsson Agency