Results 181 to 190 of about 44,908 (222)
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European Neurology, 1970
A patient with monosialoganglioside accumulation in the brain is described who shows a clinical onset of the disease at 1 year of age, no visceromegaly nor skeletal X-ray changes. There is no ganglioside accumulation in the liver. These observations permit a clear distinction from the generalized GM1 gangliosidosis and agree with the division in 2 ...
C, Hooft +3 more
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A patient with monosialoganglioside accumulation in the brain is described who shows a clinical onset of the disease at 1 year of age, no visceromegaly nor skeletal X-ray changes. There is no ganglioside accumulation in the liver. These observations permit a clear distinction from the generalized GM1 gangliosidosis and agree with the division in 2 ...
C, Hooft +3 more
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Annals of the New York Academy of Sciences, 1998
ABSTRACT: Aging is associated with the loss of brain neurotransmitter function, which apparently is the substrate for an adverse constellation of age‐associated symptoms. In particular, cholinergic deficits have been associated with cognitive impairment in aging.
M, Hadjiconstantinou, N H, Neff
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ABSTRACT: Aging is associated with the loss of brain neurotransmitter function, which apparently is the substrate for an adverse constellation of age‐associated symptoms. In particular, cholinergic deficits have been associated with cognitive impairment in aging.
M, Hadjiconstantinou, N H, Neff
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Computed tomography of GM1 gangliosidosis
The Journal of Pediatrics, 1984REPORTS DESCRIBING THE VALUE of computed tomography in the identification of white matter abnormalities in the leukodystrophies and demyelinating diseases of children first appeared in 1977.1 Since then higher resolution scanners have resulted in CT descriptions of Canavan disease, 2 Alexander disease, 3 metachromatic leukodystrophy,4. 5 Krabbe disease,
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2007
The gangliosidoses are a group of lysosomal storage diseases characterized by the accumulation of these complex glycolipids in multiple organs of the body. They manifest a predominantly neurological phenotype, a fact that is probably related to their high prevalence in nervous tissues. The typical presentation is that of a progressive neurodegenerative
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The gangliosidoses are a group of lysosomal storage diseases characterized by the accumulation of these complex glycolipids in multiple organs of the body. They manifest a predominantly neurological phenotype, a fact that is probably related to their high prevalence in nervous tissues. The typical presentation is that of a progressive neurodegenerative
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Neuropathy and anti-GM1 antibodies
Annals of Neurology, 1990Increased titers of IgM antibodies that react with carbohydrate epitopes on GM1 are present in some patients with lower motor neuron disease, sensorimotor neuropathy, or motor neuropathy with or without conduction block. Therapeutic reduction of antibody concentrations can result in clinical improvement, suggesting that the antibodies may be pathogenic.
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GM1 ganglioside and Alzheimer’s disease
Glycoconjugate Journal, 2015Assembly and deposition of amyloid ß-protein (Aß) is an invariable and fundamental event in the pathological process of Alzheimer's disease (AD). To decipher the AD pathogenesis and also to develop disease-modifying drugs for AD, clarification of the molecular mechanism underlying the Aß assembly into amyloid fibrils in the brain has been a crucial ...
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Monosialoganglioside GM1 in cerebral ischemia.
Cerebrovascular and brain metabolism reviews, 1991In vitro studies have shown that monosialoganglioside GM1 reduces excitatory amino acid-related neurotoxicity by limiting the downstream consequences of abusive excitatory amino acid receptor stimulation, while enhancing neuronotrophic factor action in a variety of neuronal cell types.
CAROLEI, ANTONIO +3 more
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