Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2001 Sep;1(5):413-21.
doi: 10.1007/s11910-001-0100-0.

Frontotemporal dementia and tauopathy

Y Yoshiyama  1 , V M Lee, J Q Trojanowski
Affiliations
Review

Frontotemporal dementia and tauopathy

Y Yoshiyama et al. Curr Neurol Neurosci Rep. 2001 Sep.

Abstract

The presence of abundant neurofibrillary lesions made of hyperphosphorylated tau proteins is the characteristic neuropathology of a subset of neurodegenerative disorders classified as "tauopathies." The discovery of mutations in the tau gene in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) constitutes convincing evidence that tau proteins play a key role in the pathogenesis of neurodegenerative disorders. Moreover, it now is known that the most common form of sporadic frontotemporal dementia (FTD), which is characterized by frontotemporal neuron loss, gliosis, and microvacuolar change, also is a tauopathy caused by a loss of tau protein expression. Thus, these discoveries have begun to change the classification and the neuropathologic diagnosis of FTD and tauopathies, as well as current understanding of the disease mechanisms underlying them. Although transgenic mice expressing wild-type human tau or variants thereof with an FTDP-17 mutation result in tau pathologies and brain degeneration similar to that seen in human tauopathies, the precise mechanisms leading to the onset and progression of neurodegenerative disorders remain incompletely understood. Here, we review current understanding of human neurodegenerative tauopathies and prospects for translative recent insights about these into therapeutic interventions to prevent or ameliorate them.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Hum Mol Genet. 1999 Apr;8(4):711-5 - PubMed
    1. Ann Neurol. 2000 Dec;48(6):939-43 - PubMed
    1. J Neurol Neurosurg Psychiatry. 1999 May;66(5):665-7 - PubMed
    1. Neurology. 1999 Mar 23;52(5):1090-3 - PubMed
    1. FEBS Lett. 1999 Mar 26;447(2-3):195-9 - PubMed

LinkOut - more resources