Niemann: Pick's Disease
Niemann Pick disease consists of a group of genetic disorders in which the common feature is a varying degree of sphingomyelin storage in certain tissues of the body. According to the current classification based on the enzymatic defect underlying these disorders, two main groups are distinguished. The first group, which comprises type A, which is characterized by a severe deficiency in acid sphingomyelinase activity, includes infantile neuronopathic form; and type B, an adult chronic form without neurologic symptoms. In the second heterogeneous group called type C, neuro-visceral involvement is massive and lipid metabolism is affected.
The sphingomyelin that accumulates in the lysosomes of the Niemann-Pick disease cells is thought to arise from the degradation of cells and their organelles since it is a major component of all mammalian cell membranes, the myelin sheath and the erythrocyte stroma. In Niemann-Pick type C, the main lipid accumulated in patients cells is not sphingomyelin but cholesterol, however, there is a close relationship between sphingomyelin metabolism and cholesterol metabolism.
Sphingomyelinase is an acidic lysosomal hydrolase that catalyses the cleavage of sphingomyelin to phosphoryl choline and ceramide. In patients with Pick’s disease its activity is deficient in all lysosome containing tissues. Patients with type A, the infantile form have 0.7% of the normal sphingomyelinase activity with median values in the range of 0-1% , while in patients with adult onset neuronopathic or non-neuronopathic disease the activity range is 0-19% of the normal, with median values in several tissues from 2-8% . This enzyme defect explains the massive deposition of sphingomyelin in tiss...
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...sh Medical Journal: 295(6610):1375-1376.
4. Levade, Salvayre, Maret and Blazy. Endogenous and Exogenous Sources of Sphingomyelinin Pick’s Disease A & B. (1988) Inher. Metab. Dis.: 11, 151-157.
5. Maziere, M. Lageron, Polonovski. Alterations in Cholesterol Metabolism in Cultured Fibroblast From Patients with N-P type C. (1987) Inher. Metab. Dis.: 10, 339-346.
6.Liscum and Faust. Low Density Lipoprotein Mediated Suppression of Cholesterol Synthesis: and LDL Uptake is Defective in N-P Type C Fibroblasts. J. Biol. Chem.: 262 (17002-17007).
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Tay-Sachs disease is a form of these lysosomal storage diseases. It is scientifically known as GM2 gangliosidosis: Hexosaminidase alpha-subunit deficiency. Three polypeptides encoded by three separate locations on the chromosome are needed for the catabolism of GM2 gangliosides. When these genes are mutated, the result is a buildup of the glycosphingolipid GM2 gangliosides. Over 50 mutations have been identified. Tay-Sachs disease is the most common form of gangliosidosis and results from a mutation of the alpha-subunit location on chromosome 15. This causes a severe dysfunction in the enzyme hexosaminidase A.
The Tuskegee Airmen changed racial perceptions by achieving goals in combat and winning important medals. They broke stereotypes by winning against their strongest enemies and destroying the tactics of these enemies. These Airmen fought many enemies in war including the Germans and they proved to many white Americans that they had the ability to fly planes in war. The Tuskegee Airmen shot down 261 German airplanes in the air and on ground (Sherman). They fought in home front battles to earn the same rights as whites, while ignoring the discrimination and segregation occurring during that time (Francis and Caso 20-21). Tuskegee pilots showed Americans that they could beat the hardest enemies that some white airmen could not. They fought for their ability to fight and to show their capabilities. They changed racial perceptions by putting up a fight to change segregation and show America that had the same rights as whites. The Tuskegee Airmen won many battles in World War II by destroying the tactics of their enemies. The Tuskegee Pilots sunk a destroyer with a machine gun and captured
The Tuskegee Airmen, also commonly referred to as Red Tails, were a group of African-American pilots who fought in World War II. These airmen were renowned for their fight against racial prejudices through their exploits in WWII. Despite of their struggles against racism they managed to prove whites mindsets wrong with their great achievements such as, never losing a single bomber under their escort to enemy fighters. Regardless of their skill, these black aviators returned to their country to find white attitudes were unchanged and joined another battle in pursuit of desegregating their military. Booker T. Washington’s philosophy of peaceful, but persistent confrontation, influenced the way Tuskegee Airmen’s challenge to confront racial barriers within the American military. Tuskegee Airmen, while simultaneously gaining the respect of whites, they also reformed of the black the image in the military.
...s Elevated in Brain of Patients with Dominantly Inherited Olivopontocerebellar Atrophy. Neuroscience Letters (submitted publication).
pathophysiology of the disease starts when the myelin sheath of both the spinal and cranial
... down defending a country that looked at him as a second class citizen. He was from Detroit and Jefferson joined the Army Air Corps in 1941. Training at Tuskegee Alabama, becoming a 2nd lieutenant in 1942. He joined one of the most decorated fighting groups in the War, flying the P-51. Based out of Italy, Jefferson flew escort missions, over Europe before he was shot down in France in 1944.
dream of going to France. He hitch-hiked from Georgia to Virginia at age eighteen. He reached Virginia, where he stowed away on a ship to Scotland, and worked his way to France. He enlisted in the French Foreign Legion immediately after the start of World War I. After being transferred to a regular unit in the French army, Bullard was wounded twice, and was declared disabled. He then applied for pilot training with a French air service and was accepted on the basis of combat heroism, thus becoming the first African American fighter pilot.
World War II opened up several opportunities for African American men during and after the war. First of all, the blacks were able to join the military, the Navy and the Army Air Corps’ (Reinhardt and Ganzel 1). The African Americans were allowed to join the military because they were needed, but they would be trained separately and put in separate groups then the white men because America was still prejudice. (Reinhardt and Ganzel 1). The same went for the African Americans that joined the Navy, only they were given the menial jobs instead of the huge jobs (Reinhardt and Ganzel 1). African Americans that joined the Army Air Corps’ were also segregated (Reinhardt and Ganzel 1). The Army Air Corps’ African American also known as the Tuskegee Airmen were sent to the blacks university in Tuskegee for their training (Reinhardt and Ganzel 1). They became one of the most well known groups of flyers during World War II th...
Before the war, African-American pilots weren't able to fly in battle due to segregation, even though blacks have been flying for a while beforehand, including pilots such as Bessie Coleman, Charles Alfred Anderson, and more, who'd fought oppression to become pilots (George 5). Army officials thought blacks couldn't fight, aren't as smart as whites, and weren't worthy enough of operating machines as complicated as airplanes. There was hope for African Americans who wanted to fly in the 1930s, when President Franklin Delano Roosevelt began to build up the U.S. armed forces, thinking of military-related ideas such as teaching civilians to fly, passing the Civilian Pilot Training Program (CPTP) in April 1939, which would provide training for 20,000 college students yearly as private pilots, and soon allowed the Secretary of War to lend equipment to schools for African-American pilot training (George 6). While this program was being developed, Edgar G. Brown, an African-American spokesman for government employees, arranged an unusual flight. Two black pilots from the National Airmen's Association, Dale White and Chauncey Spencer, would fly from Chicago, IL to Washington, D.C. in a run-down biplane. They met with Missouri senator and future president Harry S. Truman on May 9, 1939. Aviation was fairly new and a feat like this was impressive, and thus, it had impressed the president. Harry S. Truman was going to help them, and with the ...
TSEs or more commonly prion diseases are a group of invariably fatal neurodegenerative diseases that occur in humans and animals . This disease is caused by a protease –resistant protein (PrPsc) after misfolding of a host-encoded prion protein (PrP). TSEs can exist as genetic, infectious or sporadic forms. The diseases are characterized by dementia, ataxia and neuropathlogically due to loss of specific neurons in the brain. Other clinical features include persistent painful stimuli, dystonia, visual or cerebellar problems and gliosis (1).
The Tuskegee Airmen succeeded by proving success has nothing to do with color and playing a major role ending racial discrimination in the army and in the U.S. These black pilots were the first black flying unit in the U.S military. This unit was organized when Franklin D. Roosevelt decided to established an all black unit. Their first training base was located in Central Alabama near a place so called Tuskegee (Gropman). Tuskegee was located in Macon County. Not all blacks appeared to become pilots after training , but several had to become engineers, navigators, and many other varieties of occupations. In order to be a pilot, you had to be a college graduate and a possible officer in the Air Force. Many blacks from all over the U.S came to this training site to serve their country. There were multiple training phases that the men had to achieve also to be a pilot. Many men didn’t make it through all phases. After a while, they made a much larger airfield between Tallassee and Elmore County known as the new Tuskegee Army Air Field. The commander of this new airfield was a white ...
Omer, Saad B., Dr. "Vaccine Refusal, Mandatory Immunization, and the Risks of Vaccine-Preventable Diseases — NEJM." New England Journal of Medicine. Web. 13 May 2014.
9(3): 383-398. Keeney, Belea T. and Kathleen M. Heide. 1995.
This article focus on a document publishes in the Canadian Paediatric Society website, which can help council hesitant parent that refuse to vaccine their children due to safety concern. This article use research information and premeditated steps to exemplify the issue surrounding the use of vaccine on children. Research shows that health care provider has a major influence on parental decision. In addition, Doctors should take into consideration and understand parent’s specific concern, by taking the time to explain the evidence so the hesitant parents will have a better understanding and this will determine whether a child get immunize. The information that present in the article comes from the “CPS” Infectious Diseases and Immunization Committee, which is research and educational source. This article provide a clear information on what can happen if a child is not vaccinate, due to the facts that parents believe if their child is healthy and strong that they will disease free. However, most parents based their information on what they heard on the media and internet for example, that vaccine cause autism, there is no prove that it does, however things like that will make any parents not want to vaccine their child. There are consequences of a parent not having their child. In Ontario if a child is not immunize they are, not allowed in the school system, this is due to the risk that may occur. For example, a child who is vaccine, but may have a low immune system will mostly like catch whatever disease or bacteria when he encounters that specific chi...
Oscar Wilde`s novel The Picture of Dorian Gray is written primarily out of the aesthetic movement of the Nineteenth Century. Therefore, the text contains a profuse amount of imagery which reflects the concepts of beauty and sensory experiences. By taking the aesthetic approach, Wilde was able to revive the gothic style through grotesque imagery of the portrait and the character whose soul it represents. Wilde is not using gothic elements to shock his audiences; rather he uses the gothic to capture the hideousness of Gray`s corruptness which leaks out of the painting and into the tone of the entire text.