| An explanation for the changes in collagen in sporadic Amyotrophic Lateral Sclerosis. | |
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MedLine Citation:
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PMID: 21745715 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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There is evidence showing abnormalities in collagen from the skin of patients with sporadic Amyotrophic Lateral Sclerosis (sALS) both from Guam and elsewhere. The non-proteinogenic amino acid beta-N-methylamino-l-alanine (BMAA) was first associated with the high incidence of Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex (ALS/PDC) in Guam, and has been implicated as a potential environmental factor in ALS and other neurodegenerative diseases. BMAA has a number of toxic effects on motor neurons including direct agonist action on NMDA and AMPA receptors, induction of oxidative stress, and depletion of glutathione. As a non-proteinogenic amino acid, there is also the strong possibility that BMAA could cause intraneuronal protein misfolding, the hallmark of neurodegeneration. While an animal model for BMAA-induced ALS is lacking, there is substantial evidence to support a link between this toxin and ALS. We hypothesize that the abnormalities seen in sALS collagen may result from the misincorporation of BMAA and subsequent misfolding of the collagen protein. |
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Authors:
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Nicholas C Field; Tracie A Caller; Elijah W Stommel |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-7-9 |
Journal Detail:
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Title: Medical hypotheses Volume: - ISSN: 1532-2777 ISO Abbreviation: - Publication Date: 2011 Jul |
Date Detail:
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Created Date: 2011-7-12 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7505668 Medline TA: Med Hypotheses Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Elsevier Ltd. All rights reserved. |
Affiliation:
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Department of Neurology, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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