Tauopathy refers to a group of neurodegenerative diseases with pathological inclusions containing fibrillar aggregates of tau proteins. Although the tauopathies comprise a diverse phenotypic group, filamentous neuronal or neuronal and glial tau inclusions associated with the degeneration of affected brain regions are the defining neuropathological features of tauopathies (Lee et al. 2001).
Tau proteins are microtubule-associated proteins that are abundant in the central nervous system and are expressed primarily in axons. Two of the major functions of tau are to bind to and stabilize microtubules (MTs) and to promote MT polymerization. Hyperphosphorylation of tau, as observed in tauopathies, is largely thought to contribute to neuronal dysfunction and death indirectly by (1) removing tau from microtubules, which causes disruption of cellular trafficking, thus contributing to synapse dysfunction and loss and (2) making tau more fibrillogenic leading to...
References and Reading
- Ludolph, A. C., Kassubek, J., Landwehrmeyer, B. G., Mandelkow, E., Mandelkow, E. M., Burn, D. J., et al. (2009). Tauopathies with parkinsonism: Clinical spectrum, neuropathologic basis, biological markers, and treatment options. European Journal of Neurology, 16(3), 297–309.PubMedCentralCrossRefPubMedGoogle Scholar