Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016719 (Friedreich's ataxia)
 2,098 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The paper describes basic scientific and practical results obtained in the past decade at the Research Institute of Neurology, Russian Academy of Medical Sciences, in the following areas: cerebrovascular diseases, degenerative and demyelinating diseases of the nervous system. Particular attention is paid to the development of national criteria for brain death, a concept of structural and functional levels of the vascular system, that of brain pathology in atherosclerosis and essential hypertension, rapid methods for evaluating neuronal apoptosis, a new method for diagnosing prionic diseases caused by a classical viruses, and a new method for histochemical diagnosis of the degree of reparative peripheral nerve processes in polyneuropathies and to the discovery of two new hereditary nervous system diseases, to direct DNA diagnosis of torsion dystonia, Wilson-Konovalov disease, Friedreich's disease, to the setting up of a national representative family register and DNA data bank for monogenous hereditary diseases, to the development of indications for craniocerebral bypass of cerebral vessels in multiple occlusions of great cerebral arteries and indications for programmable plasmapheresis in a number of autoimmune diseases of the nervous system, to the development of rehabilitative programmes for stabilogram biocontrol, markers of an active embologenous atherosclerotic plaque in the arteries supplying blood to the brain, etc.
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PMID:[Progress in neurosciences: initiatives within the framework of the program "Brain Decade (1990-2000)"]. 1152 25

Reactive oxygen species (ROS) are produced at low levels in mammalian cells by various metabolic processes, such as oxidative phosphorylation by the mitochondrial respiratory chain, NAD(P)H oxidases, and arachidonic acid oxidative metabolism. To maintain physiological redox balance, cells have endogenous antioxidant defenses regulated at the transcriptional level by Nrf2/ARE. Oxidative stress results when ROS production exceeds the cell's ability to detoxify ROS. Overproduction of ROS damages cellular components, including lipids, leading to decline in physiological function and cell death. Reaction of ROS with lipids produces oxidized phospholipids, which give rise to 4-hydroxynonenal, 4-oxo-2-nonenal, and acrolein. The brain is susceptible to oxidative damage due to its high lipid content and oxygen consumption. Neurodegenerative diseases (AD, ALS, bipolar disorder, epilepsy, Friedreich's ataxia, HD, MS, NBIA, NPC, PD, peroxisomal disorders, schizophrenia, Wallerian degeneration, Zellweger syndrome) and CNS traumas (stroke, TBI, SCI) are problems of vast clinical importance. Free iron can react with H(2)O(2) via the Fenton reaction, a primary cause of lipid peroxidation, and may be of particular importance for these CNS injuries and disorders. Cholesterol is an important regulator of lipid organization and the precursor for neurosteroid biosynthesis. Atherosclerosis, the major risk factor for ischemic stroke, involves accumulation of oxidized LDL in the arteries, leading to foam cell formation and plaque development. This review will discuss the role of lipid oxidation/peroxidation in various CNS injuries/disorders.
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PMID:Lipid oxidation and peroxidation in CNS health and disease: from molecular mechanisms to therapeutic opportunities. 1962 72