Lewy body

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. 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. Cortical Lewy bodies are a distinguishing feature of Dementia with Lewy bodies (DLB) and 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]

Contents

History

Lewy bodies were discovered by Frederic Lewy in 1912.

Cell biology

A Lewy body is composed of the protein alpha-synuclein associated with other proteins such as ubiquitin,[4] neurofilament protein, and alpha B crystallin. Tau proteins may also be present, and Lewy bodies may occasionally be surrounded by neurofibrillary tangles.,[5][6] Lewy bodies and NFTs can occasionally exist in the same neuron, particularly the amygdala [7]

It is believed that Lewy bodies represent an aggresome response in the cell.[8]

Lewy neurites

Similarly to Lewy bodies, Lewy neurites are proteinaceous formations found in neurones of the disease brain, comprising abnormal α-synuclein filaments and granular material. They are a feature of α-synucleinopathies such as Dementia with Lewy bodies, Parkinson's disease and multiple system atrophy (MSA), and are found in the CA2-3 region of the hippocampus in Alzheimer's disease.[9]

Cultural reference

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

References

  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. 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. http://www.springerlink.com/content/03j1p2032866847h. 
  4. ^ 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. 
  5. ^ 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. Colocalization of Tau and Alpha-Synuclein Epitopes in Lewy Bodies. 
  6. ^ Arima, K; Hirai, S; Sunohara, N; Aoto, K; Izumiyama, Y; Uéda, K; Ikeda, K; Kawai, M et al. (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. 
  7. ^ 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. PMID 8740227. 
  8. ^ 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. http://www.jbc.org/cgi/pmidlookup?view=long&pmid=14627698. 
  9. ^ 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