Gangliosidosis: A Brief Review Of Associated Neuropathology Essay

Gangliosidosis: A Brief Review Of Associated Neuropathology Gangliosidosis is a lysosomal storage disease which affects primarily the nervous system. This disease is the result of an autosomal recessive mutation which causes a lack or deficiency of an enzyme important in the metabolism of gangliosides. This deficient enzyme can vary depending on the type of mutation present causing either GM1 or GM2 gangliosidosis. Each of these will be discussed later, although the overall effects are similar. Increased amounts of gangliosides inside neurons leads to, often lethal, neurodegenerative disorders. TAY- SACHS One of the more common of these disorders is known as Tay--Sachs. Bernard Sachs was the first to describe the cellular morphological features of this disease, "distended cytoplasm of the neurons and ballooning of their dendrites". Later, in the 1930’s, the term ganglioside was used to describe the accumulating material in the neurons which was characteristic of the disease. Tay-Sachs is an infantile form of GM2 gangliosidosis most often found in Ashkenazi Jews. The rate of this disease is almost ten times higher in this population than in most others. Genetically, Tay-Sachs disease (TSD) is caused by a mutation at the alpha subunit locus. In Ashkenazi Jews the mutation is primarily found in two forms. The first is a 4 base pair insertion into exon 11, the other is a G-to-C transversion mutation in the intron 12 splice site. A separate mutation has been found in the Moroccan Jews, the deletion of a phenylalanine codon in the alpha subunit sequence. Recently, other mutations have been identified in these and other populations. In all cases, these mutations impair the alpha subunit of beta hexosaminidase A resulting... ...8. Drucker, L."Identification and Rapid Detection of Three Tay--Sachs Mutations in the Moroccan Jew Population." Am. J. Human Genetics. 1992; 51: 371-77. Fredrico, A. "The Clinical Aspect of Adult Hexosaminidase Deficiencies." Developmental Neuroscience. 1991; 13: 280-287 Fukumizu, M. "Tay-Sachs Disease: Progression of Changes on Neuroimaging in Four Cases." Neuroradiology. 1992; 34: 483-486. Gravel, R.A. "Biochemistry and Genetics of Tay-Sachs Disease." The Canadian Journal of Neurological Sciences. 1991; 18: 419-423. Maia, Maria. "Juvenile GM2 Variant B1: Clinical and Biochemical Study in Seven Patients." Neuropediatrics. 1990; 21: 18-23. Specola, N. "The Juvenile and Chronic Forms of GM2 Gangliosidosis." Neurology. 1990; 40: 145-150. Suzuki, K. "Neuropathology of Late Onset Gangliosidosis." Developmental Neuroscience. 1991;13: 205-10.

Amenhotep iv & Egyptian

Amenhotep IV is an Egyptian king who is famous for introducing monotheistic religion in Egypt. Before his reign, Egyptian people worship many gods and the role of priest in their polytheistic religion. For a short period of time, he initiated a major religious revolution that affected the entire kingdom. In the early period of his reign, he changed his name from Amenhotep IV to Akhenaton. He changed his name after moving his capital to the newly designed city of Akhet-Aten. (euler. slu. edu) It depicts a major change in their religious belief.Akhenaton means â€Å"the servant of Aten†. â€Å"In the process of this religious revolution, Akhenaton placed him self as the intermediary between Aten and the people. This helped eliminate the need for the priesthood. As the only one with access to the god, Akhenaten established himself as a god-king and became the first king to be called Pharoah. † (Chesser, ehistory) The removal of â€Å"Amum† in his name is an indicati on that he is rejecting their old religion. Amum is one of their old god who is also known as god of secrets.â€Å"To have effectively removed Amun from his name seems like an all-but-open declaration of warfare against the dominant religious authority in the day, the Amun priesthood based in Thebes† (usu. edu) Akhenaten was the son of Nebmaatre Amenhotep III and Queen Tiye. He was not the oldest royal son or the heir of the throne. His older brother Prince Tuthmosis was originally the heir of the throne but he died during the reign of Amenhotep III. (euler. slu. edu) Meanwhile, his wife is the famous Nefertiti which some scholars believed as Akhenaten himself or her mother.â€Å"Evidence suggests she was wed to Akhenaten as the daughter of a high official during Amenhotep III’s reign, or of Amenhotep himself. Similarly, debate still remains as to whether or not Nefertiti was in fact the actual mother of Akhenaten, and his wife at the same time. The mysteries of Nefer titi’s origins remain a large topic of debate. † (mnsu. edu) On the other hand, it was believed that it was Nefertiti who urged Akhenaten towards religious reformation. (mnsu. edu) Religious Reformation His first step of religious reformation is moving his capital from Thebes to Akhenaten.He imposed a monotheistic religion by placing Aten as the only subject of worship. â€Å"Later, he went so far as to order the word â€Å"gods† removed and changed to â€Å"god,† wherever it occurred on public inscriptions. † (usu. edu) I was believed that the monotheistic belief of Akhenaten was inspired by Judaism through Moses or Joseph. But according to some scholars, Akhenaten belief is not purely monotheistic for the Apis cult maintained other gods. Because Akhenaten’s religious reformation was not really monotheistic, we can’t conclude that it was influenced by Judaism.(ancientegyptonline. co. uk) As part of his religious reformation, he opened Egyptian religion for all instead of being exclusive for Egyptian priest. The reason why he rejected Amun as god is because he is the god of secrets. Instead of worshipping a god of secret, he wanted the Egyptian people to worship a god like Aten which reflects its presence through the rays of sun. (usu. edu) Mark Damen described Akhetaten’s god as follows: â€Å"The religious iconography of Akhenaten's new belief system centered around the aten as a divine presence.Representing the life-giving force of the universe, the sun-disk is often depicted in either abstract or personified form, occasionally both at the same time. Though it's most often pictured as a mere circle with rays of light radiating downward, the aten also appears sometimes with little hands appended onto the end of its solar beams holding out to worshipers the ankh, the Egyptian sign of life. In a few instances, the hands are even shoving the ankh rather unceremoniously up the noses of the blessed, a figura tive assertion, no doubt, that the sun offers the â€Å"breath of life.† It would seem less comical today if this sacrament didn't look so much like an incontinent ear-swab. † (usu. edu) Although Akhenaten desired the entire Egptian people to have a relationship with Aten, still the said relationship became exclusive for Akhenaten and his family. Instead of worshipping Aten directly, they just worship the royal family. (ancientegyptonline. co. uk) â€Å"All this concurs well with Akhenaten's religion, where the pharaoh was said to serve as the conduit between humanity and the Aten. In other words, it's through and because of him the sun-disk bestows life on the planet.In his own words, a hymn Akhenaten claims to have composed himself about the Aten† (usu. edu) One reason for this is the torturing heat of the sun in Egypt. One way of worshipping is standing under its presence and basking in its radiance. On the other hand, priests during his reign did not like his religious reformation. His own imposed religion disregard the significance of priest in their religious life for Akhenaten served as a priest. Threats in his kingdom During the Amama period, a widespread of famine and diseases affected the Egyptians. Scholars believed that a plague or influenza killed thousands of lives during that time.I was also believed that that was the same plague in the book of Exodus that there is no sufficient evidence for that. (ancientegyptonline. co. uk) Egyptians viewed this plague as a consequence of neglecting their other gods. Death According to archeologist, there are indications that Akhenaten died in his middle age. Although the cause of his death is still unknown, there are assumptions that he died because of skin cancer. (usu. edu) As part of his religious duty, exposed himself to the torturous heat of the Egyptian sun that might caused him that disease. References Akhenaten and Monotheism.Usu. edu. Febraury 6 2008 Ancient Egypt Online. FEbraur y 6 2008 Bart, Anneke. Ancient Egypt. Euler. slu. edu Febraury 6 2008 Nefertiti: Queen of Dynasty XVII. Mnsu. edu. February 6 2008 Preston, Chester. E History Archive. February 6 2008

Bf Skinner - 1166 Words

Burrhus Frederic Skinner the Theorist Behaviorism is the branch of psychology associated today with numerous psychologists but one of the most prominent behavioral psychologists of all time, B.F. Skinner really taught people that any behavior is usually immediately affected by its consequences. I having a young child I have seen Skinners theories work in many different facets during my short stint of being a parent. Skinner is a theorist who made his reputation by studying how an individuals behavior might change by responding to his/her environment. The great Skinner wrote Education is what survives when what has been learned has been forgotten,(Skinner, 1964) thus basically meaning that most over and over again behaviors we as†¦show more content†¦His most commonly known product was his use of Skinners Box,( Kerry W. Buckley,1989) This box contraption was used as an study aide for most of his research that I discussed above. Skinners Box showed how ones behavior is changed due to there charted research, eithe r via special rewards and/or unexpected punishment. This contraption Skinners Box was basically a small box that a rat or another small animal was put into to be viewed during research. As the animal went through many different scientific test, it would receive rewards and punishments, such as food pellets /or electric shock, in response to the choice of the animals actions. All of these actions were documented and studied providing us with many of Skinners behavioral processes such as schedules of reinforcement, shaping and aversive stimuli. All of these methods are still found to be used in one way or another by therapists today. In mental institutions and prisons therapist often still use Skinners ideas to break criminal patterns or mental issues. In the prison system if you do good you are rewarded with points or tokens, these tokens are used to buy you the necessary things in order to survive, the downside if you mess up these same tokens or points are taken away from you makin g it difficult to survive in the volatile environment. Unfortunately these methods are looked for by the same institution members once outside and the everyday world doesnt provide these same opportunities.Show MoreRelatedBehavioral Psychology and BF Skinner916 Words   |  4 PagesThere was never a man more influential to Behavioral psychology than B.F. Skinner. He was well-acknowledged for his theories in behaviorism and remains as one of the best and most controversial figures in the world of psychology. Most of his work throughout his lifetime dealt with both positive and negative reinforcements and the after-effects it had on behavior. B.F. Skinner understood that as humans, everything that we do or become is through the methods of rewards and punishments. Though his timeRead MoreThe Theory Of Operant Conditioning And The Skinner Box1707 Words   |  7 PagesThere have been many famous individuals who have contributed to education. BF Skinner, William Glasser, John Dewey, and Lev Vygotski are four notorious men in the history and impact of education. Bf Skinner (1904) was a behaviorist who his most widely known for his theory of operant conditioning and the Skinner box. His created this theory to study human behavior. Operant conditioning includes positive and negative reinforcement as well as positive and negative punishment. Positive reinforcementRead MoreEssay on Reinforcement Versus Goal Theory724 Words   |  3 Pagesincreasing motivation and efforts, but also through increasing and improving the feedback quality. 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