Myelin is a lipid-rich extramembranous structure found on the axons and dendrites of neuron in many bilaterian animals, mainly vertebrates, as well as some arthropods and annelids. A circumferential wrapping of myelin, known as a myelin sheath, increases the conduction speed of electrical impulses passing along the axon by generating saltatory conductions, which are much faster than conduction along an unmyelinated axon, and also reduce signal loss due to extrinsic disturbances.
Adrenoleukodystrophy (ALD) is a disease linked to the X chromosome. It is a result of fatty acid buildup caused by failure of peroxisomal fatty acid beta oxidation which results in the accumulation of very long chain fatty acids in tissues throughout the body. The most severely affected tissues are the myelin in the central nervous system, the adrenal cortex, and the Leydig cells in the testes. The long chain fatty acid buildup causes damage to the myelin sheath of the neurons of the brain, resulting in seizures and hyperactivity. Other symptoms include problems in speaking, listening, and understanding verbal instructions.
Canavan disease, or Canavan–Van Bogaert–Bertrand disease, is a rare and fatal autosomal recessive degenerative disease that causes progressive damage to nerve cells and loss of white matter in the brain. It is one of the most common degenerative cerebral diseases of infancy. It is caused by a deficiency of the enzyme aminoacylase 2, and is one of a group of genetic diseases referred to as leukodystrophies. It is characterized by degeneration of myelin in the phospholipid layer insulating the axon of a neuron and is associated with a gene located on human chromosome 17.
Pelizaeus–Merzbacher disease is an X-linked neurological disorder that damages oligodendrocytes in the central nervous system. It is caused by mutations in proteolipid protein 1 (PLP1), a major myelin protein. It is characterized by a decrease in the amount of insulating myelin surrounding the nerves (hypomyelination) and belongs to a group of genetic diseases referred to as leukodystrophies.
Mantua is a census-designated place (CDP) in Fairfax County, Virginia, United States. Mantua is a bedroom community serving as a suburb to the Washington, D.C. metropolitan area. Most of the homes in Mantua were built between the 1950s and the 1980s. The population was 7,503 at the 2020 census.
John Davison Rockefeller III was an American philanthropist. Rockefeller was the eldest son and second child of John D. Rockefeller Jr. and Abby Aldrich Rockefeller as well as a grandson of Standard Oil co-founder John D. Rockefeller. He was engaged in a wide range of philanthropic projects, many of which his family had launched, as well as supporting organizations related to East Asian affairs. Rockefeller was also a major supporter of the Population Council, and the committee that created the Lincoln Center in Manhattan.
Lorenzo's oil is a liquid solution made of 4 parts glycerol trioleate and 1 part glycerol trierucate, which are the triacylglycerol forms of oleic acid and erucic acid. It is prepared from olive oil and rapeseed oil. It is used in the investigational treatment of asymptomatic patients with adrenoleukodystrophy (ALD), a nervous system disorder.
Lorenzo's Oil is a 1992 American drama film directed and co-written by George Miller. It is based on the true story of Augusto and Michaela Odone, parents who search for a cure for their son Lorenzo's adrenoleukodystrophy (ALD), leading to the development of Lorenzo's oil. The film was shot in Pittsburgh, Pennsylvania, primarily from September 1991 to February 1992. It had a limited release in North America on December 30, 1992, with a nationwide release two weeks later, on January 15, 1993. Though it was a box office disappointment, grossing $7.2 million against its $30 million budget, the film was generally well received by the critics and garnered two nominations at the 65th Academy Awards.
Zellweger syndrome is a rare congenital disorder characterized by the reduction or absence of functional peroxisomes in the cells of an individual. It is one of a family of disorders called Zellweger spectrum disorders which are leukodystrophies. Zellweger syndrome is named after Hans Zellweger (1909–1990), a Swiss-American pediatrician, a professor of pediatrics and genetics at the University of Iowa who researched this disorder.
Krabbe disease (KD) is a rare and often fatal lysosomal storage disease that results in progressive damage to the nervous system. KD involves dysfunctional metabolism of sphingolipids and is inherited in an autosomal recessive pattern. The disease is named after the Danish neurologist Knud Krabbe (1885–1961).
Leukodystrophies are a group of, usually, inherited disorders, characterized by degeneration of the white matter in the brain. The word leukodystrophy comes from the Greek roots leuko, "white", dys, "abnormal" and troph, "growth". The leukodystrophies are caused by imperfect growth or development of the glial cells which produce the myelin sheath, the fatty insulating covering around nerve fibers. Leukodystrophies may be classified as hypomyelinating or demyelinating diseases, respectively, depending on whether the damage is present before birth or occurs after. Other demyelinating diseases are usually not congenital and have a toxic or autoimmune cause.
Points of Light is an international nonprofit, nonpartisan organization headquartered in Georgia, United States dedicated to engaging more people and resources in solving serious social problems through voluntary service.
Metachromatic leukodystrophy (MLD) is a lysosomal storage disease which is commonly listed in the family of leukodystrophies as well as among the sphingolipidoses as it affects the metabolism of sphingolipids. Leukodystrophies affect the growth and/or development of myelin, the fatty covering which acts as an insulator around nerve fibers throughout the central and peripheral nervous systems. MLD involves cerebroside sulfate accumulation. Metachromatic leukodystrophy, like most enzyme deficiencies, has an autosomal recessive inheritance pattern.
The National Multiple Sclerosis Society (NMSS) is a nonprofit organization founded in 1946.
Augusto Daniel Odone and Michaela Teresa Murphy Odone were the parents of Lorenzo Michael Murphy Odone, a boy with the illness adrenoleukodystrophy (ALD). They became famous for developing a controversial treatment using Lorenzo's oil for their son's incurable illness. This quest was recounted in the film Lorenzo's Oil (1992). Augusto had previously been an economist for the World Bank.
Hugo Wolfgang Moser (1924–2007) was a Swiss-born American research scientist and director of the Neurogenetics Research Center at the Kennedy Krieger Institute. Moser was also University Professor of Neurology and Pediatrics at Johns Hopkins University. His research on peroxisomal disorders achieved international recognition.
The Kennedy Krieger Institute is a nonprofit, 501(c)(3) tax-exempt, Johns Hopkins affiliate located in Baltimore, Maryland, that provides in-patient and out-patient medical care, community services, and school-based programs for children and adolescents with learning disabilities, as well as disorders of the brain, spinal cord, and musculoskeletal system. The Institute provides services for children with developmental concerns mild to severe and is involved in research of various disorders, including new interventions and earlier diagnosis.
Arati Prabhakar is an American engineer and public official. Since October 3, 2022, she has served as the 12th director of the White House Office of Science and Technology Policy and Science Advisor to the President.
Tunku Tan Sri Dato' Seri Imran ibni Almarhum Tuanku Ja’afar is a Malaysian sports administrator. He was born as the second son of Ja'afar of Negeri Sembilan, the elected monarch of Negeri Sembilan, one of the thirteen states of Malaysia. He is often known in English simply as Prince Tunku Imran.
The TerraMar Project was a self-described environmental nonprofit organization. It was founded in 2012 in the United States by convicted sex offender Ghislaine Maxwell. A sister organisation in the United Kingdom was incorporated in 2013. TerraMar (U.S.) announced its closure on 12 July 2019. This was shortly after New York federal prosecutors arrested Maxwell's close associate Jeffrey Epstein. Epstein was a financier who was being charged with sex trafficking crimes for a second time. TerraMar (UK) was officially dissolved on 3 December 2019.
