Lewy body
From Wikipedia, the free encyclopedia
Lewy bodies are abnormal aggregates of protein that develop inside nerve cells. They are identified under the microscope when histology is performed on the brain.
Lewy bodies appear as spherical masses that displace other cell components. There are two morphological types: classical (brain stem) Lewy bodies and cortical Lewy bodies. A classical Lewy body is an eosinophilic cytoplasmic inclusion that consists of a dense core surrounded by a halo of 10-nm wide radiating fibrils, the primary structural component of which is alpha-synuclein. In contrast, a cortical Lewy body is less well defined and lacks the halo. Nonetheless, it is still made up of alpha-synuclein fibrils.
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[edit] Cell biology
A Lewy body is composed of the protein alpha-synuclein associated with other proteins such as ubiquitin,[1] neurofilament protein, and alpha B crystallin.
It is believed that Lewy bodies represent an aggresome response in the cell.[2]
[edit] Associated diseases
Lewy bodies are associated with several conditions:
- Parkinson's disease[3][4]
- dementia with Lewy bodies [5]
- the Lewy body variant of Alzheimer's disease
- Hallervorden-Spatz syndrome[6]
Inclusions composed of alpha synuclein, different from Lewy bodies, can be found in glial cells in multiple system atrophy. These are termed glial cytoplasmic inclusions. Multiple system atrophy can be clinically confused with Parkinson's disease.
[edit] History
Lewy bodies were first seen and linked to Parkinson's disease ("paralysis agitans") in 1912 by the neurologist Frederic Lewy (1885-1950).[7]
[edit] References
- ^ Engelender S (April 2008). "Ubiquitination of alpha-synuclein and autophagy in Parkinson's disease". Autophagy 4 (3): 372–4. PMID 18216494. http://www.landesbioscience.com/journals/auto/abstract.php?id=5604.
- ^ Tanaka M, Kim YM, Lee G, Junn E, Iwatsubo T, Mouradian MM (February 2004). "Aggresomes formed by alpha-synuclein and synphilin-1 are cytoprotective". J. Biol. Chem. 279 (6): 4625–31. doi:. PMID 14627698. http://www.jbc.org/cgi/pmidlookup?view=long&pmid=14627698.
- ^ P. Duffy and V. Tennyson. Phase and electron microscopic observations of Lewy bodies and melanin granules in the substantia nigra and locus coeruleus in Parkinson's disease. J. Neuropathol. Exp. Neurol. 24: 398-414, 1965
- ^ Jellinger KA (2009). "Significance of brain lesions in Parkinson disease dementia and Lewy body dementia". Front Neurol Neurosci 24: 114–25. doi:. PMID 19182469. http://content.karger.com/produktedb/produkte.asp?doi=10.1159/000197890&typ=pdf.
- ^ Gomperts SN, Rentz DM, Moran E, et al (September 2008). "Imaging amyloid deposition in Lewy body diseases". Neurology 71 (12): 903–10. doi:. PMID 18794492. PMC: 2637553. http://www.neurology.org/cgi/pmidlookup?view=long&pmid=18794492.
- ^ Zarranz JJ, Gómez-Esteban JC, Atarés B, Lezcano E, Forcadas M (January 2006). "Tau-predominant-associated pathology in a sporadic late-onset Hallervorden-Spatz syndrome". Mov. Disord. 21 (1): 107–11. doi:. PMID 16114023. http://dx.doi.org/10.1002/mds.20661.
- ^ E. Forster and F. H. Lewy. "Paralysis agitans". In Pathologische Anatomie. Handbuch der Neurologie (edited by M. Lewandowsky). Berlin: Springer Verlag 1912, 920-933.
[edit] External links
- Lewy Body Dementia Association, Inc. The Lewy Body Dementia Association (LBDA) is a U.S. based 501(c)(3) nonprofit organization dedicated to raising awareness of the Lewy body dementias (LBD), supporting patients, their families and caregivers, and promoting scientific advances. The Association's purposes are charitable, educational, and scientific.
- Lewy Body Journal
- Lewy Body Dementia (in english)(en Françes) information from the Alzheimer Society of Canada (in english)(en Françes)
- Lewy Body Society - The UK's only Lewy Body charity.
- Too violent to hold, too sick not to Chicago Tribune article of one individual with the affliction.
- Alzheimer's Society factsheet - What is dementia with Lewy bodies?
