PMID: 9160282Apr 1, 1997Paper

Ascorbate availability and neurodegeneration in amyotrophic lateral sclerosis

Medical Hypotheses
A B Kok

Abstract

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease in which upper and lower motoneurons progressively deteriorate and die. Neuronal damage is most evident in the lower central nervous system, and death generally occurs following central respiratory failure. Proposed and demonstrated mechanisms for amyotrophic lateral sclerosis are diverse, and include altered superoxide dismutase and neurofilament proteins, autoimmune attack, and hyperglutamatergic activity. However, they do not account for the late onset of the disease, its earlier onset in males, and the differential vulnerability of neurons located in the brainstem and spinal cord. It is proposed here that, within the context of a specific defect such as altered superoxide dismutase, age-dependent decline in ascorbate availability triggers the disease. A role for ascorbate, which is found in millimolar levels in neurons, is suggested by a number of consistencies: 1) superoxide radicals being a common substrate for superoxide dismutase and ascorbate; 2) a close association between central nervous system ascorbate levels and injury tolerance; 3) a steady decline in ascorbate plasma levels and cellular availability with age; 4) plasma ascorbate levels being lowe...Continue Reading

References

Jul 1, 1979·Physiological Reviews·B ChanceA Boveris
Jun 16, 1977·The New England Journal of Medicine·R Spector
Jul 1, 1979·Annals of Neurology·H M Kurlander, B M Patten
Oct 1, 1978·Archives of Neurology·A D Rosen
Jan 1, 1992·Free Radical Biology & Medicine·S KongI Fridovich
Dec 10, 1992·The New England Journal of Medicine·R G SmithS H Appel
Jan 1, 1992·EXS·J C Monboisse, J P Borel
Sep 1, 1992·Mutation Research·R S Sohal, U T Brunk
Nov 21, 1992·Annals of the New York Academy of Sciences·L AmaducciA Lippi
Jun 1, 1992·Proceedings of the National Academy of Sciences of the United States of America·A JainA Meister
May 1, 1992·Annals of Neurology·K HisanagaF R Sharp
Aug 1, 1992·Neurology·R W HubbardS A Tan
Jan 1, 1991·Journal of Neural Transmission. Parkinson's Disease and Dementia Section·E SoficK Jellinger
Aug 1, 1991·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·B R Brooks
Sep 1, 1991·Trends in Neurosciences·D L CookW F Hopkins
Oct 1, 1991·Biological Trace Element Research·M A SerraE Sabbioni
Feb 14, 1992·Archives of Biochemistry and Biophysics·C M Dhanbhoora, J R Babson
Mar 1, 1991·Journal of the Neurological Sciences·P A Sillevis SmittM A Kuipers
Mar 1, 1991·Journal of the Neurological Sciences·M E Shils
Nov 1, 1990·Neuroreport·M D Majewska, J A Bell
Feb 1, 1991·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·L H ZengT W Wu
Jan 1, 1990·Free Radical Research Communications·B Halliwell
Jun 8, 1990·Neuroscience Letters·L HilleredU Pontén
Oct 1, 1988·Trends in Neurosciences·M P Blaustein
Jan 1, 1989·Free Radical Research Communications·R T DeanM A McElligott
Jan 1, 1989·Acta Neuropathologica·R M GarrutoD C Gajdusek
Jul 1, 1989·Acta Physiologica Scandinavica·T Eken, O Kiehn
Jun 9, 1988·Biochimica Et Biophysica Acta·R C Rose
Jan 1, 1987·Annals of the New York Academy of Sciences·U Moser

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