Neuronal loss, neurofibrillary tangles and granulovacuolar degeneration in the hippocampus with ageing and dementia

1977 ◽  
Vol 37 (2) ◽  
pp. 111-118 ◽  
Author(s):  
M. J. Ball
2007 ◽  
Vol 10 (2) ◽  
pp. 3-14 ◽  
Author(s):  
M Ozansoy ◽  
A Başak

Tauopathies: A Distinct Class of Neurodegenerative DiseasesNeurodegenerative diseases are characterized by neuronal loss and intraneuronal accumulation of fibrillary materials, of which, neurofibrillary tangles (NFT) are the most common. Neurofibrillary tangles also occur in normal aging and contain the hyperphosphorylated microtubule-associated protein tau. A detailed presentation is made of the molecular bases of Alzheimer's disease (AD), postencephalitic parkinsonism, amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of Guam, progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Pick's disease, frontotemporal dementia (FTD), Down's syndrome, myotonic dystrophy (DM) and Niemann-Pick Type C (NPC) disease, which are considered to be common tauopathies. The unique human tau gene extends over 100 kb of the long arm of chromosome 17 and contains 16 exons. The human brain contains six tau isoforms that contain from 352 to 441 amino acids. To date, 34 pathogenic tau mutations have been described among 101 families affected by FTD with parkinsonism linked to chromosome 17 (FTDP-17). These mutations may involve alternative splicing of exon 10 that lead to changes in the proportion of 4-repeat- and 3-repeat-tau isoforms, or may modify tau interactions with microtubules. Tau aggregates differ in degree of phosphorylation and in content of tau isoforms. Five classes of tauopathies have been defined depending on the type of tau aggregates. The key event in tauopathies is the disorganization of the cytoskeleton, which is based on mutations/polymorphisms in the tau gene and lead to nerve cell degeneration. In this review, tauopathies as a distinct class of neurodegenerative diseases are discussed with emphasis on their molecular pathology and genetics.


1995 ◽  
Vol 89 (4) ◽  
pp. 291-295 ◽  
Author(s):  
P. Cras ◽  
Mark A. Smith ◽  
Peggy L. Richey ◽  
Sandra L. Siedlak ◽  
Paul Mulvihill ◽  
...  

2019 ◽  
Vol 139 (3) ◽  
pp. 463-484 ◽  
Author(s):  
Marta J. Koper ◽  
Evelien Van Schoor ◽  
Simona Ospitalieri ◽  
Rik Vandenberghe ◽  
Mathieu Vandenbulcke ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Kristen E. Funk ◽  
Jeff Kuret

Alzheimer's disease is characterized pathologically by extracellular senile plaques, intracellular neurofibrillary tangles, and granulovacuolar degeneration. It has been debated whether these hallmark lesions are markers or mediators of disease progression, and numerous paradigms have been proposed to explain the appearance of each lesion individually. However, the unfaltering predictability of these lesions suggests a single pathological nidus central to disease onset and progression. One of the earliest pathologies observed in Alzheimer's disease is endocytic dysfunction. Here we review the recent literature of endocytic dysfunction with particular focus on disrupted lysosomal fusion and propose it as a unifying hypothesis for the three most-studied lesions of Alzheimer's disease.


2015 ◽  
Vol 7 (1) ◽  
Author(s):  
Sally Hunter ◽  
◽  
Thais Minett ◽  
Tuomo Polvikoski ◽  
Elizabeta Mukaetova-Ladinska ◽  
...  

1995 ◽  
Vol 89 (4) ◽  
pp. 291-295 ◽  
Author(s):  
Patrick Cras ◽  
Mark A. Smith ◽  
Peggy L. Richey ◽  
Sandra L. Siedlak ◽  
Paul Mulvihill ◽  
...  

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