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Topic: Neurodegenerative disease

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	The Institute of Neurology, Queen Square, London, UK
		The Department of Neurodegenerative Disease’s research portfolio encompasses prion diseases (predominantly within the embedded MRC Prion Unit), Alzheimer’s disease and related disorders, Huntington’s disease, motor neurone disease and frontotemporal dementia and studies of the pathways of cellular senescence.
		The research philosophy is to combine basic and clinical translational research; many of the key contributions towards understanding the basic biology of these diseases have come from clinical and neuropathological observations, and efficient translation of basic laboratory research to the clinic is essential to provide benefits for patients at the earliest opportunity.
		Prion diseases have long been known to be able to cross the species barrier and this has been supported by key laboratory experiments.
	www.ion.ucl.ac.uk /research/neurodegen_dis/neurodegen_intro.htm (800 words)

	Hospital Practice: Neurodegenerative Disease and Cancer (Site not responding. Last check: 2007-11-04)
		Neural cell death is abnormally accelerated in such neurodegenerative diseases as those of the Alzheimer's, Huntington's, Parkinson's, Kennedy's (X-linked spinal and bulbar muscular atrophy), and Machado-Joseph (Azorean) types.
		While development of actual cures could prove elusive--in the case of the neurodegenerative diseases, therapeutic options are currently few--simply postponing clinical onset could have an impressive effect on mortality and allow the oldest segment of our population to enjoy a much longer period of productivity.
		Kennedy's disease: Caspase cleavage of the androgen receptor is a crucial event in cytotoxicity.
	www.hosppract.com /issues/2001/09/bred.htm (1776 words)

	Michigan Parkinson Foundation (Site not responding. Last check: 2007-11-04)
		Neurodegenerative Disease: A disease in which nerve cells in the brain or spinal cord (central nervous system, CNS) progressively die or degenerate.
		Pathology of Parkinson's disease: Examination of brain tissue from Parkinson's disease patients under the microscope shows loss of the dark-colored dopamine-producing nerve cells in the substantia nigra pars compacta and appearance of Lewy bodies, abnormal small round clumps of protein and other materials which are rarely seen except in Parkinson's disease.
		The tremor of Parkinson's disease is called a "resting tremor" because it is present when a limb is at rest and may be reduced or go away when the limb is held up or otherwise used by the patient.
	www.parkinsonsmi.org /facts.htm (1100 words)

	Neurodegenerative disease in children might respond to neurosteroids
		While the treatment did not target the cause of the disease -- a mutation in one of two genes that disrupts the transport of cholesterol within all cells of the body -- nor cure it, the therapy substantially delayed the onset of weight loss, motor coordination, mobility -- and death.
		If synthesis of these steroid hormones is found to be disrupted in other neurodegenerative diseases -- a question that has just begun to be investigated -- neurosteroid therapy could prove effective in stalling them, she says.
		NP-C is an autosomal recessive disease, meaning both parents must contribute a copy of the mutated gene to cause the disease, and that there is a 25 percent chance their offspring will develop it.
	www.eurekalert.org /pub_releases/2004-07/uoc--ndi070904.php (1161 words)

	Parkinson's Disease: An Overview
		Parkinson's disease is a common neurodegenerative disease of the elderly.
		The diagnosis of Parkinson's disease is a clinical exercise.
		The difficulty in accurately distinguishing between neurodegenerative diseases that have Parkinsonian extrapyramidal features (multiple system atrophy, progressive supranuclear palsy (PSP), etc.) is reflected in statistics showing a high rate of misdiagnosis among movement disorder experts when patients are followed throughout the course of their illness to actual autopsy.
	www.clevelandclinicmeded.com /diseasemanagement/neurology/parkinsons/parkinsons.htm (2901 words)

	Neurodegenerative Disease Targeted in UK Government's Drive for New Medical Treatments (Site not responding. Last check: 2007-11-04)
		This is the latest development in the Government's drive to help the advancement of new medical treatments in six specific disease areas - stroke, diabetes, medicines for children, mental health, cancer, and dementias and neurodegenerative diseases.
		Parkinson's disease is a devastating illness that affects all aspects of life for those with the condition and current treatments can cause severe side effects.
		For more information on the Parkinson's Disease Society, the charity dedicated to supporting all people with Parkinson's, their families, friends and carers, visit http://www.parkinsons.org.uk or call the national help line on 0808 800 0303 or contact the press office on 0207 963 9370.
	www.medicalnewstoday.com /medicalnews.php?newsid=30828 (1271 words)

	Research in Progress, AE28
		Sporadic Creutzfeldt-Jakob (sCJD) disease is caused by the mutation or spontaneous conversion of the normal to the abnormal form of the prion protein.
		Almost all neurodegenerative diseases are the result of malfunctioning proteins in nerve cells or an inability to process these proteins, causing them to build up to dangerous levels in the brain.
		To treat both prion-related and other neurodegenerative diseases, researchers are trying to develop drugs that target the buildup of proteins: beta-amyloid plaques in Alzheimer’s and CJD and huntingtin protein aggregation in HD.
	www.stanford.edu /group/hopes/rltdsci/inprogress/ae28.html (562 words)

	Center for Neurodegenerative Disease (Site not responding. Last check: 2007-11-04)
		Welcome to the Emory Center for Neurodegenerative Disease, where research cuts across traditional academic departments and scientific disciplines to focus on mechanisms of neurodegeneration, neuroprotection and brain repair.
		Adding to the urgency of the problem is the fact that the incidence of these age-related disorders is increasing rapidly as population demographics change.
		The research spans from genetic and environmental factors that cause disease, to development of new diagnostic and therapeutic approaches, to clinical testing of new treatments.
	www.neurology.emory.edu /CND (162 words)

	Understanding Neurodegenerative Disease
		At the present time, significant headway in the study of neurodegenerative disease is being made.
		There now is substantial evidence for the involvement of each EAA receptor subtype in one or more human neurodegenerative syndrome, and recent findings suggest that EAA receptors are sensitive mediators of excitotoxicity at both ends of the age spectrum."(231) All forms of MSG are exogenous sources of glutamic acid.
		Coyle, J.T. Glutamate receptors and age-related neurodegenerative disorders.
	www.truthinlabeling.com /UnderstandingNeurodegen.html (419 words)

	Yeast Point to Multiple Mutations in Neurodegenerative Disease (Site not responding. Last check: 2007-11-04)
		The causes of most human diseases have been difficult to pinpoint because they are likely to involve defects in multiple genes.
		Huntington’s disease is caused by mutations in the huntingtin gene, but other gene mutations may affect the age of onset and severity of disease.
		Muchowski’s work on Huntington’s disease caught the attention of Susan Lindquist and Outeiro, both of the Whitehead Institute for Biomedical Research in Cambridge, Massachusetts, who were studying the role of the α-synuclein gene in Parkinson’s disease.
	www.genomenewsnetwork.org /articles/12_03/yeast_screen.shtml (666 words)

	Amyloid diseases: Abnormal protein aggregation in neurodegeneration -- Koo et al. 96 (18): 9989 -- Proceedings of the ...
		Transthyretin is a tetrameric protein that is the backup transporter of thyroxine and the main transporter of the retinol-binding protein (carries 20% of vitamin A) (14).
		Typically, the familial diseases have a much earlier age of onset (30 vs. 80 yr), and the patients present with peripheral neuropathy and/or organ dysfunction as a result of massive transthyretin amyloid deposition.
		Transthyretin amyloid diseases are protein-misfolding diseases where the normally folded tetrameric protein dissociates to an alternatively folded monomer that is capable of self-assembly into amyloid fibrils (17, 18).
	www.pnas.org /cgi/content/full/96/18/9989 (3019 words)

	Northwestern University move closer to new therapies for neurodegenerative disease
		What is known about Lou Gehrig's disease, as it is commonly called, is that misfolded and damaged proteins clump together in cells to form aggregates and motor neurons die.
		In the study, published this month in the Journal of Cell Biology, the scientists looked one at a time at neuronal cells expressing the mutant SOD1 protein and found that in cells where the protein accumulated and aggregates formed, 90 percent of the cells went on to die.
		For the other 90 percent the disease is not the result of one mutation but rather a series of many genetic events that debilitate motor neurons.
	www.news-medical.net /?id=14086 (717 words)

	Epidemiology 2003; 14(4):413-419
		We studied all economically active individuals in the Swedish 1980 census (4,812,646 subjects), and followed them for neurodegenerative disease mortality from 1981 through 1995.
		An increased risk of Alzheimer's disease mortality was observed among men exposed both in 1970 and 1980 (relative risk = 2.3; 95% confidence interval = 1.6-3.3 for exposure ≥0.5 μT).
		The associations were most pronounced for early-onset Alzheimer's disease mortality or with follow-up limited to 10 years after the last known occupation.
	www.studiosra.it /news/occupational.htm (307 words)

	NIH Guide: XENOBIOTICS AND CELL DEATH/INJURY IN NEURODEGENERATIVE DISEASE
		RESEARCH OBJECTIVES Much of the previous research in neurodegenerative diseases and dysfunction has focused on the underlying biological processes critical to disease manifestation in the hopes of developing new treatments; however, the relative roles of environmental, endogenous neurochemical and genetic factors in the cause of neurodegenerative diseases remain unclear.
		Attempts are needed to understand the contribution of environmental exposure, endogenous susceptibility factors, and increasing age in the etiopathology of neurodegenerative disease and will require a concurrent advancement and refinement of methodologies and sciences.
		Studies on the effect of aging on toxicant-induced neurodegeneration, since aging is a risk factor for a number of neurodegenerative diseases.
	grants.nih.gov /grants/guide/pa-files/PAS-99-054.html (1720 words)

	NEJM -- Alzheimer's Disease and Parkinson's Disease (Site not responding. Last check: 2007-11-04)
		Secreted amyloid beta-protein similar to that in the senile plaques of Alzheimer's disease is increased in vivo by the presenilin 1 and 2 and APP mutations linked to familial Alzheimer's disease.
		Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease.
		A susceptibility gene for late-onset idiopathic Parkinson's disease.
	content.nejm.org /cgi/content/full/348/14/1356 (4578 words)

	NIH Guide: GENE/ENVIRONMENT INTERACTION IN NEURODEGENERATIVE DISEASE
		Recent research in these diseases has focused on both the underlying biological processes critical to disease manifestation for the development of new treatments and on the relative roles of environmental, endogenous neurochemical and genetic factors in their etiologies.
		In previous years, the NIEHS has focused its efforts on the expansion of research in Parkinson's disease culminating in the establishment of Collaborative Centers for Parkinson's Disease Environmental Research (See http://www.niehs.nih.gov/dert/consorti.htm) This new initiative will selectively shift its research focus each year to emphasize a different neurodegenerative disease.
		The disease commonly strikes in the fifth through seventh decades of life, although cases in young adults and in the elderly are known.
	grants1.nih.gov /grants/guide/pa-files/PAS-03-160.html (3291 words)

	E. Coli 0157 - The Main Cause of Neurodegenerative Disease? (Site not responding. Last check: 2007-11-04)
		Based upon the compelling demographic correlations, the apparent pathophysiological links, and the positive preliminary experimental results of three prospective treatments for neurodegenerative diseases, it is proposed that E. coli 0157:H7 and other Shiga toxin-producing E. coli have a primary role in the pathogenesis of the diseases for which positive correlations have been found.
		Criteria for Rating Correlation Strength (pg 57) Neurodegenerative Diseases with Positive Correlations to E. coli (pg 58) Diseases with Strong Positive Correlations (pg 58) Diseases with Positive Correlations (pg 58) Diseases with Moderately Positive Correlations (pg 58) Table 3 Neurodegenerative Diseases with Correlation E. Coli 0157:H7 (pg 59) Figure 15.
		Non-Neurodegenerative Diseases with Correlation to E. Coli 0157:H7 (pg 73) Diseases with Negative Correlations to E. Coli (pg 74) Strong Negative Correlations (pg 74) Negative Correlations (pg 74) Figure 27.
	www.ecoli0157.com /summary.html (1534 words)

	Parkinson's Disease -- familydoctor.org (Site not responding. Last check: 2007-11-04)
		Parkinson's disease is a disorder that causes a progressive loss of nerve cell function in the part of the brain that controls muscle movement.
		People with Parkinson's disease experience tremors (shakiness) as a result of the damage to their nerve cells.
		The tremor of Parkinson's disease gets worse when the person is at rest and better when the person moves.
	familydoctor.org /handouts/187.html (371 words)

	Memory in Neurodegenerative Disease - Cambridge University Press
		They present the neuroanatomical and neurochemical basis of memory disorders in neurodegenerative disease, and review the contribution of neuroradiology and neuropathology to the understanding of memory and amnesia.
		The impact of depression on memory in neurodegenerative disease Julie A. Fields, Suzanne Norman, Kristy A. Straits-Tröster, Alexander I. Tröster; 20.
		Surgical interventions in neurodegenerative disease: impact on memory and cognition Steven B. Wilkinson, Alexander I. Tröster; 23.
	www.cup.cam.ac.uk /catalogue/catalogue.asp?isbn=0511038453 (659 words)

	[No title] (Site not responding. Last check: 2007-11-04)
		Krabbe disease is a degenerative neurological disorder primarily affecting infants and young children, although rare cases of adult onset have been described.
		Disease progression is generally rapid, leading to death within 1-2 years.
		The disease is inherited as an autosomal recessive trait caused by mutations in the galactocerebrosidase (GALC) gene that significantly reduce the activity of the enzyme.
	www.mayo.edu /res-jax/EckmanResearch/EckmanResearch.html (564 words)

	Welcome to MIND
		A common thread links these illnesses that together affect millions of Americans: there are no cures, nor are there effective treatments that consistently slow or stop the relentless course of these devastating neurodegenerative diseases.
		The MassGeneral Institute for Neurodegenerative Disease (MIND) is now poised to change the future for people with Alzheimer’s, ALS (Lou Gehrig’s), Huntington’s, Parkinson’s and other neurodegenerative diseases.
		The MassGeneral Institute for Neurodegenerative Disease’s model of collaboration and sharing of resources will hasten the pace of discovery and, we hope, change the prognosis for these diseases in this generation.
	www.mghmind.org (214 words)

	The genetic epidemiology of neurodegenerative disease -- Bertram and Tanzi 115 (6): 1449 -- Journal of Clinical ...
		Familial Alzheimer’s disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer’s disease type 3 gene.
		Estimation of the genetic contribution of presenilin-1 and -2 mutations in a population-based study of presenile Alzheimer disease.
		Association of late-onset Alzheimer disease with a genotype of PLAU, the gene encoding urokinase-type plasminogen activator on chromosome 10q22.2.
	www.jci.org /cgi/content/full/115/6/1449 (6190 words)

	Understanding Neurodegenerative Disease
		At the present time, significant headway in the study of neurodegenerative disease is being made.
		There now is substantial evidence for the involvement of each EAA receptor subtype in one or more human neurodegenerative syndrome, and recent findings suggest that EAA receptors are sensitive mediators of excitotoxicity at both ends of the age spectrum."(231) All forms of MSG are exogenous sources of glutamic acid.
		Coyle, J.T. Glutamate receptors and age-related neurodegenerative disorders.
	www.truthinlabeling.org /UnderstandingNeurodegen.html (419 words)

	Alzheimer's Disease and Neurodegenerative Disorders
		This program focuses on Alzheimer's Disease and related dementias.
		The staff consist of neurologists, psychiatrists and nurses with experience and expertise in these disorders.
		This short video shows changes that occur to the brain from Alzheimer's disease.
	www.umm.edu /neurosciences/alzheimer.html (119 words)

	Neurodegenerative Disease In Children Might Respond To Neurosteroids
		Mutations In A Multifunctional Protein Cause Parkinsonism (December 2, 2004) -- A team of researchers at Mayo Clinic in Jacksonville, Fla., and colleagues in Canada and Germany have discovered a gene and six mutations of it that cause symptoms associated with Parkinson's...
		Study Finds Autoimmune Link In Juvenile Batten Disease (May 23, 2002) -- For years, researchers have tried to determine how the defective gene in juvenile Batten disease leads to the seizures, mental impairment, and other symptoms of this devastating childhood disorder.
		Zebrafish And CHIP Help Untangle Protein Misfolding In Brain Disease (October 31, 2005) -- Protein handling is especially important for neurons because damage or death of brain cells causes neurological disease.
	www.sciencedaily.com /releases/2004/07/040713082347.htm (1365 words)

	eMedicine - Menkes Disease : Article by Celia H Chang, MD (Site not responding. Last check: 2007-11-04)
		A girl with the Menkes disease phenotype and an X:autosome chromosomal translocation was described in 1987, which led to the identification of the locus on the X chromosome in 1993.
		The Wilson disease gene (WND) is expressed predominantly in liver, whereas the Menkes disease gene (MNK) is not expressed in liver.
		Menkes disease is caused by mutations of the Xq13.3 gene (ATP7A).
	www.emedicine.com /neuro/topic569.htm (2531 words)

	Parkinsons Disease 1 (Site not responding. Last check: 2007-11-04)
		Parkinson's disease (PD) is characterized by an insidious onset with slowing of emotional and voluntary movement, muscular rigidity, postural abnormality and tremor.
		Parkinson's disease was first described in 1817 by James Parkinson in a paper entitled "An Essay on the Shaking Palsy" (photos courtesy of the National Library of Medicine).
		The disease is due to the striatal deficiency of dopamine following neuronal degeneration within the substantia nigra.
	medweb.bham.ac.uk /http/depts/clin_neuro/teaching/tutorials/parkinsons/parkinsons1.html (1799 words)

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