Lewy body

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Photomicrographs of regions of substantia nigra in this Parkinson's patient show Lewy bodies and Lewy neurites in various magnifications. Top panels show a 60-times magnification of the alpha-synuclein intraneuronal inclusions aggregated to form Lewy bodies. The bottom panels are 20x magnification images that show strand-like Lewy neurites and rounded Lewy bodies of various sizes. Neuromelanin-laden cells of the substantia nigra are visible in the background. Stains used: mouse monoclonal alpha-synuclein antibody; counterstained with Mayer's haematoxylin
Lewy bodies

Lewy bodies are abnormal aggregates of protein that develop inside nerve cells in Parkinson's disease (PD), Lewy body dementia and some other disorders. They are identified under the microscope when histology is performed on the brain.

Lewy bodies appear as spherical masses that displace other cell components. The two morphological types are classical (brain stem) Lewy bodies and cortical Lewy bodies. A classical Lewy body is an eosinophilic cytoplasmic inclusion consisting 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. Cortical Lewy bodies are a distinguishing feature of Dementia with Lewy bodies (DLB), but may occasionally be seen in ballooned neurons characteristic of Pick's disease and corticobasal degeneration,[1] as well as in patients with other tauopathies.[2] They are also seen in cases of multiple system atrophy, particularly the Parkinsonian variant.[3]

History[edit]

Lewy bodies were discovered by Frederic Lewy in 1912.

Cell biology[edit]

Photomicrograph of the dorsal motor nucleus of the vagus nerve (DmX) in a transverse section along the upper medulla shown to be affected by the abnormally deposited alpha synuclein as part of intraneuronal Lewy bodies found (extreme right): DmX is one of the earliest sites affected by synuclein deposition in Parkinson's disease.[4]

A Lewy body is composed of the protein alpha-synuclein associated with other proteins, such as ubiquitin,[5] neurofilament protein, and alpha B crystallin. Tau proteins may also be present, and Lewy bodies may occasionally be surrounded by neurofibrillary tangles (NFTs).[6][7] Lewy bodies and NFTs can occasionally exist in the same neuron, particularly in the amygdala.[8]

Lewy bodies are believed to represent an aggresome response in the cell.[9]

Lewy neurites[edit]

Lewy neurites are abnormal neurites in diseased neurons, containing granular material and abnormal α-synuclein filaments similar to those found in Lewy bodies.[10] Like Lewy bodies, lewy neurites are a feature of α-synucleinopathies such as dementia with Lewy bodies, Parkinson's disease and multiple system atrophy (MSA).[11] They are also found in the CA2-3 region of the hippocampus in Alzheimer's disease.[11]

Cultural reference[edit]

Kelsey Grammer's lead character, mayor of Chicago Tom Kane in the television series Boss, suffers from early-stage dementia with Lewy bodies.

See also[edit]

References[edit]

  1. ^ Dickson DW, Feany MB, Yen SH, Mattiace LA, Davies P. (1996). "Cytoskeletal pathology in non-Alzheimer degenerative dementia: new lesions in diffuse Lewy body disease, Pick's disease, and corticobasal degeneration". Journal of Neural Transmission. Supplementum 47: 31–46. doi:10.1007/978-3-7091-6892-9_2. PMID 8841955. 
  2. ^ Popescu, A; Lippa, CF; Lee, VM; Trojanowski, JQ (2004). "Lewy Bodies in the Amygdala: Increase of -Synuclein Aggregates in Neurodegenerative Diseases With Tau-Based Inclusions". Archives of Neurology 61 (12): 1915–1919. doi:10.1001/archneur.61.12.1915. PMID 15596612. 
  3. ^ Jellinger KA (2007). "More frequent Lewy bodies but less frequent Alzheimer-type lesions in multiple system atrophy as compared to age-matched control brains". Acta Neuropathologica 114 (3): 299–303. doi:10.1007/s00401-007-0227-4. PMID 17476513. 
  4. ^ Braak H, Del Tredici K, Rüb U, de Vos RA, Jansen Steur EN and Braak E (March–April 2003). "Staging of brain pathology related to sporadic Parkinson's disease.". Neurobiol Aging 24 (2): 197–211. doi:10.1016/S0197-4580(02)00065-9. PMID 12498954. 
  5. ^ Engelender S (April 2008). "Ubiquitination of alpha-synuclein and autophagy in Parkinson's disease". Autophagy 4 (3): 372–4. PMID 18216494. 
  6. ^ Ishizawa, Takashi MD; Matilla, Petri MD, PhD; Davies, Peter PhD; Wang, Dengshun MD; Dickson, Dennis W. MD (April 2003). [Colocalization of Tau and Alpha-Synuclein Epitopes in Lewy Bodies "Colocalization of tau and alpha-synuclein epitopes in Lewy bodies."]. Journal of Neuropathology & Experimental Neurology 62 (4): 389–397. PMID 12722831. 
  7. ^ Arima, K; Hirai, S; Sunohara, N; Aoto, K; Izumiyama, Y; Uéda, K; Ikeda, K; Kawai, M; Arima K, Hirai S, Sunohara N, Aoto K, Izumiyama Y, Uéda K, Ikeda K, Kawai M. (1999). "Cellular co-localization of phosphorylated tau- and NACP/alpha-synuclein-epitopes in Lewy bodies in sporadic Parkinson's disease and in dementia with Lewy bodies". Brain Research 843 (1–2): 53–61. doi:10.1016/S0006-8993(99)01848-X. PMID 10528110. 
  8. ^ Marie Luise Schmidt, John A. Martin, Virginia M.-Y. Lee, John Q. Trojanowski (1996). "Convergence of Lewy bodies and neurofibrillary tangles in amygdala neurons of Alzheimer's disease and Lewy body disorders". Acta Neuropathol 91 (5): 475–81. doi:10.1007/s004010050454. PMID 8740227. 
  9. ^ 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:10.1074/jbc.M310994200. PMID 14627698. 
  10. ^ Maria Grazia Spillantini, R. Anthony Crowther, Ross Jakes, Masato Hasegawa, Michel Goedert (26 May 1998). "α-Synuclein in filamentous inclusions of Lewy bodies from Parkinson’s disease and dementia with Lewy bodies". PNAS 95 (11): 6469–73. PMC 27806. PMID 9600990. 
  11. ^ a b Wami Marui, Eizo Iskei, Masanori Kato, Hiroyasu Akatsu, Kenji Kosaka (2004). "Pathological entity of dementia with Lewy bodies and its differentiation from Alzheimer's disease". Acta Neuropathologica 108 (2): 121–8. doi:10.1007/s00401-004-0869-4. PMID 15235805. 

External links[edit]