Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nervous system tissues from a number of patients with idiopathic neurological disorders were examined for biochemical evidence of RNA tumor virus infection. RNase-sensitive DNA polymerase activity was found in a cytoplasmic particulate fraction from two patients with Guamanian amyotrophic lateral sclerosis (ALS) but not in brains from two normal U.S. individuals. The buoyant density of the enzyme-containing fraction was 1.16-1.18 g/ml and could be converted to a denser region of the gradient (1.24 g/ml) by treatment with the nonionic surfactant, Sterox. The cation and detergent requirements for the endogenous RNase-sensitive DNA polymerase reaction were determined. The early (5 min) endogenous reverse transcriptase product was analyzed by cesium sulfate gradient centrifugation. RNase- and heat-sensitive RNA-DNA hybrids were detected in the product analysis of two ALS, one Parkinsonism-dementia (PD) brain, and two brains from asymptomatic Chamorros but not in brains from normal U.S. individuals and a number of patients with neuro-psychiatric disorders. The DNA product was a 4.5S heteropolymer that hybridized more extensively to RNA extracted from the enzyme-containing pellet from PD brain as compared to a similar fraction from normal U.S. brain. The DNA product appeared to be unrelated to Rausvher or visna virus 70S RNA as determined by RNA-[-3H]DNA hybridization.
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PMID:RNA-instructed DNA polymerase activity in a cytoplasmic particulate fraction in brains from Guamanian patients. 4 90

Jejunal mucosa from 7 patients with amyotrophic lateral sclerosis (A.L.S.), 20 newly reported patients with multiple sclerosis (M.S.), and 35 control patients without either disease was studied by immunofluorescence. An immune reaction was present in all A.L.S. specimens and consisted of altered ratios of immunoglobulin-labelled cells in the lamina propria, complement-labelled cells in the same location, and, in some, immunoglobulin and complement deposits in the epithelial basement membrane. Poliovirus antigen was detected in 4 cases, and in 1 of the these cases measles antigen was also present. A fifth specimen showed large amounts of herpesvirus antigen. In 2 cases studied at necropsy, both viral infection and immunological change was confined to the proximal jejunum. Measles antigen was identified in every case of M.S., and in biopsy specimens from 16 of the 20 M.S. patients immunological reactions similar to those seen in A.L.S. were present. With 2 exceptions, the controls did not show these changes in the jejunal mucosa. The exceptions were a patient with Friedreich's ataxia, who had an increase of IgG-labelled cells and some complement-bearing cells in the lamina propria, and a patient diagnosed as having non-tropical sprue, in whom large quantities of herpes antigen were seen.
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PMID:Jejunal immunopathology in amyotrophic lateral sclerosis and multiple sclerosis. Identification of viral antigens by immunofluorescence. 6 22

Cells from explants and monolayer subcultures of adult human brain obtained from biopsies or autopsies of ten multiple sclerosis (MS) cases, one case each of Jakob-Creutzfeld (JC) and amyotrophic lateral sclerosis (ALS) and three cases with no central nervous system (CNS) involvement were transformed with PML-SV40 virus. Transformation was effected to establish permanent lines of these particular adult brain cells so that sufficient quantities would be available for other research projects. The procedure previously used to transform human fibroblasts (Koprowski et al., '62) was successfully applied to human brain cells. The success of transformation was dependent on the growth condition of the cultures at the time of infection. Events occurring after viral infection and during the pre-transformation and the post-transformation phases are described.
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PMID:Human brain in tissue culture. III. PML-SV40-induced transformation of brain cells and establishment of permanent lines. 16 35

Using radial immunodiffusion, albumin and immunoglobulin G were determined in non-preconcentrated cerebrospinal fluid from 127 controls and from 239 patients. In controls the concentrations of albumin and immunoglobulin G followed normal distribution. The two variables were correlated linearly (r = 0.60). The elliptic bivariate normal range was calculated, and was found to contain 95% of the paired values. As a clinical limit, this range discriminated more effectively between normal and altered pairs than the two one-dimensional normal ranges X+/-2 s, thus improving the evaluation of laboratory findings in the single case. Likewise in clinically defined groups of patients, bivariate evaluation of results provided additional evidence. In many distinct clinical syndromes, e.g. bacterial encephalomeningitis, polyneuropathy, amyotrophic lateral sclerosis, albumin and immunoglobulin G concentrations exhibited an especially close correlation, probably resulting from damage to the blood-cerebrospinal fluid barrier. However, no correlation of these two variables was detected in acute encephalomeningitis due to virus infection, and in multiple sclerosis: in these groups, immunoglobulin G concentrations were elevated independently of albumin. Since evidence is lacking as to the pathogenesis of multiple sclerosis, it seems noteworthy that the same phenomenon was observed in a well-defined group of viral infections.
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PMID:[Bivariate evaluation of laboratory findings: immunoglobulin G and albumin in cerebrospinal fluid (author's transl)]. 96

The cause of amyotrophic lateral sclerosis is unknown. In this review clinical and scientific data that are pertinent to understanding this disease are reviewed. There are currently several major controversies concerning the possible role of immunological factors, genetic factors, environmental toxins, and viral infection in pathogenesis. These concepts must be considered in relation to what is known about the disease in all its aspects, including epidemiological data, information on the classical and molecular pathology of the disease, and on associated involvement of other systems, e.g., the spinocerebellar pathways and frontal dementia. Only when all this information is assimilated can full understanding of the disease and, hopefully, a logical approach to treatment and prevention, be achieved.
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PMID:What do we really know about amyotrophic lateral sclerosis? 133 40

The causes of the neurodegenerative disorders of Parkinson's disease (PD), Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS) are unknown. It is proposed that all these disorders result primarily from a loss of trophic peptidergic neurotransmitter, possibly Substance P (SP). This loss in turn produces the classical neuronal degeneration seen in each of these diseases and occurs due to a combination of natural aging and chronic autoimmune destruction following a viral infection of the CNS, early in life. The loss is therefore slow and by the time of clinical presentation the inflammatory process is disappearing as the antigenic stimulus lessens with its removal. The implications of the theory in terms of future research and therapy are briefly discussed.
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PMID:Substance P and neurodegenerative disorders. A speculative review. 172 84

The causes of human amyotrophic lateral sclerosis (ALS) and the spinal muscular atrophies (SMA) are, almost without exception, unknown. This ignorance has stimulated the search for animal models to obtain insight into the etiology, pathogenesis and biochemical mechanisms underlying the human disorders. None of the 38 animal models, described in this review, provides an exact animal copy of a specific human motor neuron disease. Most of the models reproduce certain structural or physiological aspects of their human counterparts. The various experimental models can be classified according to the pathogenetic mechanism involved and according to the structural changes observed. Models based on experimentally induced disease, include heavy metals and trace elements (lead intoxication in guinea pigs, rabbits, rats, cats and primates; mercury intoxication in rats; aluminium intoxication in rabbits; swayback in goat kids; calcium and magnesium deficient rabbits and primates and calcium deficient cynomolgus monkeys), toxins (IDPN, vincristine, vinblastine, podophyllotoxin, colchicine, maytansine, maytanprine, L-BMAA, lectins, adriamycin), nutritional factors (ascorbic acid deficient guinea pigs), virus infection (spongiform polioencephalomyelitis, attenuated poliovirus, lactate dehydrogenase-elevating virus), and immunological factors (immunization with motor neurons). Hereditary models comprise hereditary canine spinal muscular atrophy, hereditary neurogenic amyotrophy in the pointer dog, Stockard paralysis, Swedish Lapland dog paralysis, "wobbler" mouse, "shaker" calf, and hereditary spinal muscular atrophy in zebra foals, crossbred rabbits,
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PMID:Animal models of amyotrophic lateral sclerosis and the spinal muscular atrophies. 267 Dec 67

The epidemiological aspects of amyotrophic leukospongiosis (AL), a slow viral infection of the central nervous system leading to the fatal outcome in 2-4 years, have been studied. As a rule, this disease is observed in the inhabitants of rural areas or in town dwellers born in rural areas and having spent there a considerable part of their life. AL occurs in persons of middle and older age; young people under 19 years and old people over 68 years of age are not affected by this infection. In contrast to amyotrophic lateral sclerosis and the Jakob-Creutzfeldt disease, AL is characteristic for persons in the phase of hormonal activity. The disease starts mostly in autumn and winter; this regularity is especially pronounced in women. The morbidity level (according to the average annual data) is at present 0.3 per million of population. An increased morbidity rate is characteristic of the family and group type of the epidemic process. This higher morbidity rate, by one order higher than that observed in the sporadic type of morbidity, is caused by the gradual formation of "genetic isolates".
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PMID:[Epidemiological research in amyotrophic leukospongiosis]. 296 Nov 73

The cause of amyotrophic lateral sclerosis is not known. However, there is increasing evidence that the syndrome has more than one cause. Many of the different categories of causation are dealt with in the papers presented in this symposium - including genetic and acquired forms, and multiple categories within those two major classes. The diversity of different causes gives hope that progress in one field will be followed by elucidation of other fields. As examples, there has been progress in understanding the genetic forms due to hexosaminidase deficiency and molecular genetics is being applied to other heritable motor neuron diseases. Among acquired diseases, in different parts of the world, there has been new evidence that persistent viral infection may be important, that dietary constituents may be important, and that paraproteinemia may be important.
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PMID:Research progress in motor neuron diseases. 306 74

The pathogenesis of EMC virus induced diabetes has generally been thought to be caused by direct cytopathic effect of the virus on beta cells with susceptibility or resistance dictated primarily by the density of viral receptors on the beta cells of different individuals. The histological finding of insulitis, our demonstration of a protective effect of immunosuppression with ALS or anti-theta antibody and silica supports host immune factors as important determinants of susceptibility. A critical role of the immune system might be mediated by autoimmune destruction of EMC-virus infected beta cells. In susceptible strain mice treated with low dose cyclophosphamide to deplete suppressor cells, which may halt the autoimmune process and allow recovery, a prolonged period of hyperglycemia was demonstrated as compared to controls. Bone marrow exchanged between susceptible and resistant strains was also found to alter susceptibility. "B mice", deficient in T lymphocytes, when infected with EMC virus had a decreased incidence of diabetes. Susceptibility to EMC diabetes may be dictated by the autoaggressive response of host immune system to beta cells altered by EMC virus infection.
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PMID:The importance of immunologic factors in the pathogenesis of encephalomyocarditis virus induced diabetes in mice. 609 11


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