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)

Emerging evidence suggests that thiamine deficiency (TD), the cause of Wernicke's encephalopathy, produces alterations in brain function and structural damage that closely model a number of maladies in which neurodegeneration is a characteristic feature, including Alzheimer's disease, amyotrophic lateral sclerosis, Parkinson's disease, multiple sclerosis, along with alcoholic brain disease, stroke, and traumatic brain injury. Impaired oxidative metabolism in TD due to decreased activity of thiamine-dependent enzymes leads to a multifactorial cascade of events in the brain that include focal decreases in energy status, oxidative stress, lactic acidosis, blood-brain barrier disruption, astrocyte dysfunction, glutamate-mediated excitotoxicity, amyloid deposition, decreased glucose utilization, immediate-early gene induction, and inflammation. This review describes our current understanding of the basis of these abnormal processes in TD, their interrelationships, and why this disorder can be useful for our understanding of how decreased cerebral energy metabolism can give rise to cell death in different neurodegenerative disease states.
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PMID:Modeling neurodegenerative disease pathophysiology in thiamine deficiency: consequences of impaired oxidative metabolism. 2113 Aug 21

Neuroglia is critically important for controlling the brain homeostasis and for mounting the brain defence against pathological insults. Here, we overview recent data about the role of neuroglia in various types of neurodegenerative diseases (Alzheimer's disease, Parkinson's disease, fronto-temporal dementia, Wernicke encephalopathy, amyotrophic lateral sclerosis and immunodeficiency virus-1-associated dementia). In all these forms of neurodegeneration, astroglia undergoes complex morphological and functional changes. The early and mid-term stages of neurodegenerative processes, and specifically of Alzheimer's disease, are associated with generalised atrophy of astroglia, whereas the later stages are characterised with an astrogliosis and microglial activation linked to neuropathological lesions such as senile plaques. Atrophic changes in astroglia may contribute to the initial cognitive deficits due to reduced glial synaptic coverage and decreased neuroprotection.
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PMID:Neuroglial roots of neurodegenerative diseases? 2116 12