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Title: Tauopathy  
Author: World Heritage Encyclopedia
Language: English
Subject: Biochemistry of Alzheimer's disease, Dementia, Parkinson's disease, Alzheimer's disease, Infantile progressive bulbar palsy
Collection: Cytoskeletal Defects, Dementia, Histopathology, Medical Signs
Publisher: World Heritage Encyclopedia


Classification and external resources
MeSH D024801
Diagram of a normal microtubule and one affected by tauopathy

Tauopathies are a class of neurodegenerative diseases associated with the pathological aggregation of tau protein[1] in the human brain.

The best-known of these illnesses is Alzheimer's disease (AD), wherein tau protein is deposited within neurons in the form of neurofibrillary tangles (NFTs). They were first described by the eponymous Alois Alzheimer in one of his patients suffering from the disorder. Tangles are formed by hyperphosphorylation of a microtubule-associated protein known as tau, causing it to aggregate in an insoluble form. (These aggregations of hyperphosphorylated tau protein are also referred to as PHF, or "paired helical filaments"). The precise mechanism of tangle formation is not completely understood, and it is still controversial as to whether tangles are a primary causative factor in the disease or play a more peripheral role. AD is also classified as an amyloidosis because of the presence of senile plaques.[2]

The degree of NFT involvement in AD is defined by Braak stages. Braak stages I and II are used when NFT involvement is confined mainly to the transentorhinal region of the brain, stages III and IV when there's also involvement of limbic regions such as the hippocampus, and V and VI when there's extensive neocortical involvement. This should not be confused with the degree of senile plaque involvement, which progresses differently.[3]

Other conditions in which neurofibrillary tangles are commonly observed include:

In Pick's disease and corticobasal degeneration tau proteins are deposited in the form of inclusion bodies within swollen or "ballooned" neurons.[14]

Argyrophilic grain disease (AGD), another type of dementia,[15][16][17] is marked by the presence of abundant argyrophilic grains and coiled bodies on microscopic examination of brain tissue.[18] Some consider it to be a type of Alzheimer disease.[18] It may co-exist with other tauopathies such as progressive supranuclear palsy and corticobasal degeneration,[2] and also Pick's disease.[19]

Huntington's disease: a neurodegenerative disease caused by a CAG tripled expantion in huntingtin gen is the most recently describred Taupahty (Fernandez-Nogales et al Nat Med 2014). JJ Lucas and co-workers demosntrated that in HD brains tau levels are increased and that the 4R/3R balance is altered. In addition, in this study, JJ Lucas shows intranuclear isoluble deposits of tau. These "Lucas rods" were also found in Alzheimer's disease brains.

Some other tauopathies include:

The non-Alzheimer's tauopathies are sometimes grouped together as "Pick's complex".[20][21][22]

See also


  1. ^ Rizzo, G.; Martinelli, P.; Manners, D.; Scaglione, C.; Tonon, C.; Cortelli, P.; Malucelli, E.; Capellari, S. et al. (2008). "Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and Parkinson's disease". Brain 131 (Pt 10): 2690–700.  
  2. ^ a b c Dickson, DW (2009). "Neuropathology of Non-Alzheimer Degenerative Disorders". International journal of clinical and experimental pathology 3 (1): 1–23.  
  3. ^ Braak, H.; Braak, E. (1991). "Neuropathological stageing of Alzheimer-related changes". Acta Neuropathologica 82 (4): 239–59.  
  4. ^ Williams, David R; Lees, Andrew J (2009). "Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges". The Lancet Neurology 8 (3): 270–9.  
  5. ^ Roberts, GW (1988). "Immunocytochemistry of neurofibrillary tangles in dementia pugilistica and Alzheimer's disease: evidence for common genesis". Lancet 2 (8626–8627): 1456–8.  
  6. ^ Selkoe, Dennis J.; Podlisny, Marcia B. (2002). "Deciphering the genetic basis of Alzheimer's disease". Annual Review of Genomics and Human Genetics 3: 67–99.  
  7. ^ Hof, P. R.; Nimchinsky, E. A.; Bu�e-Scherrer, V.; Bu�e, L.; Nasrallah, J.; Hottinger, A. F.; Purohit, D. P.; Loerzel, A. J. et al. (1994). "Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders". Acta Neuropathologica 88 (5): 397–404.  
  8. ^ Santa-Maria, Ismael; Haggiagi A; Liu X; Wasserscheid J; Nelson PT; Dewar K; Clark LN; Crary JF (Nov 2012). "The MAPT H1 haplotype is associated with tangle-predominant dementia". Acta Neuropathologica 124 (5): 693–704.  
  9. ^ Jellinger, K. A.; Attems, J. (2006). "Neurofibrillary tangle-predominant dementia: comparison with classical Alzheimer disease". Acta Neuropathologica 113 (2): 107–17.  
  10. ^ Brat, Daniel J.; Gearing, Marla; Goldthwaite, Patricia T.; Wainer, Bruce H.; Burger, Peter C. (2001). "Tau-associated neuropathology in ganglion cell tumours increases with patient age but appears unrelated to ApoE genotype". Neuropathology and Applied Neurobiology 27 (3): 197–205.  
  11. ^ Halper, J; Scheithauer, BW; Okazaki, H; Laws Jr, ER (1986). "Meningio-angiomatosis: a report of six cases with special reference to the occurrence of neurofibrillary tangles". Journal of neuropathology and experimental neurology 45 (4): 426–46.  
  12. ^ Paula-Barbosa, M. M.; Brito, R.; Silva, C. A.; Faria, R.; Cruz, C. (1979). "Neurofibrillary changes in the cerebral cortex of a patient with subacute sclerosing panencephalitis (SSPE)". Acta Neuropathologica 48 (2): 157–60.  
  13. ^ Wisniewski, Krystyna; Jervis, George A.; Moretz, Roger C.; Wisniewski, Henryk M. (1979). "Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia". Annals of Neurology 5 (3): 288–94.  
  14. ^ Arai, Tetsuaki; Ikeda, Kenji; Akiyama, Haruhiko; Shikamoto, Yasuo; Tsuchiya, Kuniaki; Yagishita, Saburo; Beach, Thomas; Rogers, Joseph et al. (2001). "Distinct isoforms of tau aggregated in neurons and glial cells in brains of patients with Pick's disease, corticobasal degeneration and progressive supranuclear palsy". Acta Neuropathologica 101 (2): 167–73.  
  15. ^ Ferrer I, Santpere G, van Leeuwen FW (2008). "Argyrophilic grain disease".  
  16. ^ Josephs KA, Whitwell JL, Parisi JE, Knopman DS, Boeve BF, Geda YE, Jack CR Jr, Petersen RC, Dickson DW (2008). "Argyrophilic grains: a distinct disease or an additive pathology?".  
  17. ^ Wallon, D.; Sommervogel, C.; Laquerrière, A.; Martinaud, O.; Lecourtois, M.; Hannequin, D. (2010). "Maladie des grains argyrophiles : composante synergique de la démence ?" [Argyrophilic grain disease: synergistic component of dementia?].  
  18. ^ a b Tolnay, M; Monsch, AU; Staehelin, HB; Probst, A (1999). "Argyrophilic grain disease: differentiation from Alzheimer disease". Der Pathologe 20 (3): 159–68.  
  19. ^ Jellinger KA (1998). "Dementia with grains (argyrophilic grain disease".  
  20. ^ Kertesz, Andrew (2003). "Pick Complex: An Integrative Approach to Frontotemporal Dementia". The Neurologist 9 (6): 311–7.  
  21. ^ Kertesz, Andrew (2003). "Pick's complex and FTDP-17". Movement Disorders 18: 57–62.  
  22. ^ Andrew Kertesz, Paul McMonagle, Mervin Blair, Wilda Davidson and David G. Munoz (July 20, 2005). "The evolution and pathology of frontotemporal dementia". Brain 128 (9): 1996–2005.  

External links

  • Delacourte, A (2005). "Tauopathies: recent insights into old diseases". Folia neuropathologica 43 (4): 244–57.  
  • Buée, L; Delacourte, A (1999). "Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease". Brain Pathology 9 (4): 681–93.  
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