Neurofibrillary tangle
Neurofibrillary tangles are pathological protein aggregates found within neurons in cases of Alzheimer's disease. 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 whether tangles are a primary causative factor in the disease or play a more peripheral role.
Other conditions in which neurofibrillary tangles are commonly observed include:
- Progressive supranuclear palsy[1] although with straight filament rather than PHF tau
- Dementia pugilistica (chronic traumatic encephalopathy)[2]
- Frontotemporal dementia and parkinsonism linked to chromosome 17 however without detectable β-amyloid plaques.[3]
- Lytico-Bodig disease (Parkinson-dementia complex of Guam)[4]
- Ganglioglioma and gangliocytoma[5]
- Meningioangiomatosis [6]
- Subacute sclerosing panencephalitis[7]
- As well as lead encephalopathy, tuberous sclerosis, Hallervorden-Spatz disease, and lipofuscinosis[8]
References
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- Pathologic page about Neurofibrillary tangles, by the University of Oklahoma.
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id:Filamen PH it:Ammassi neurofibrillari pl:Splątki neurofibrylarne
ru:Нейрофибриллярный клубок- Jump up ↑ Williams DR, Lees AJ. (2009)Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol.8:270-9.
- Jump up ↑ Roberts GW. (1988) Immunocytochemistry of neurofibrillary tangles in dementia pugilistica and Alzheimer's disease. Lancet.2:1456-8.
- Jump up ↑ Selkoe DJ, Podlisny MB (2002) Deciphering the genetic basis of Alzheimer's disease. Annu Rev Genomics Hum Genet 3: 67-99.
- Jump up ↑ Hof PR, et al.(1994) Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. Acta Neuropathol. 88:397-404.
- Jump up ↑ Brat DJ, Gearing M, Goldthwaite PT, Wainer BH, Burger PC. (2001) Tau-associated neuropathology in ganglion cell tumours increases with patient age but appears unrelated to ApoE genotype. Neuropathol Appl Neurobiol 27:197-205
- Jump up ↑ Halper J, Scheithauer BW, Okazaki H, Laws ER Jr. (1986) Meningio-angiomatosis. J Neuropathol Exp Neurol. 1986 Jul;45(4):426-46.
- Jump up ↑ Paula-Barbosa MM, Brito R, Silva CA, Faria R, Cruz C.(1979) Neurofibrillary changes in the cerebral cortex of a patient with subacute sclerosing panencephalitis (SSPE). Acta Neuropathol 48:157-60
- Jump up ↑ Wisniewski K, Jervis GA, Moretz RC, Wisniewski HM.(1979) Alzheimer neurofibrillary tangles in diseases other than senile and presenile dementia.Ann Neurol 5:288-94
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