Presenile appearance of abundant Alzheimer's neurofibrillary tangles without senile plaques in the brain in myotonic dystrophy

1991 ◽  
Vol 82 (1) ◽  
pp. 1-5 ◽  
Author(s):  
A. Kiuchi ◽  
N. Otsuka ◽  
Y. Namba ◽  
I. Nakano ◽  
M. Tomonaga
Keyword(s):  
Myotonic Dystrophy ◽  
Neurofibrillary Tangles ◽  
Senile Plaques ◽  
Alzheimer's Neurofibrillary Tangles ◽  
The Brain

Immunohistochemical investigation of the brain of aged dogs. I. Detection of neurofibrillary tangles and of 4-hydroxynonenal protein, an oxidative damage product, in senile plaques

Amyloid ◽  
2001 ◽  
Vol 8 (1) ◽  
pp. 11-21 ◽  
Author(s):  
Nikolaos Papaioannou ◽  
Peter C.J. Tooten ◽  
Anne Marie van Ederen ◽  
Jurgen R.E. Bohl ◽  
Jaime Rofina ◽  
...  
Keyword(s):  
Oxidative Damage ◽  
Neurofibrillary Tangles ◽  
Senile Plaques ◽  
Immunohistochemical Investigation ◽  
The Brain

Neurofibrillary tangles and deposition of oxidative products in the brain in cases of myotonic dystrophy

2006 ◽  
Vol 26 (2) ◽  
pp. 107-114 ◽  
Author(s):  
Reiko Oyamada ◽  
Masaharu Hayashi ◽  
Yuji Katoh ◽  
Kuniaki Tsuchiya ◽  
Toshio Mizutani ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Neurofibrillary Tangles ◽  
Oxidative Products ◽  
The Brain

The binding of basic fibroblast growth factor to Alzheimer's neurofibrillary tangles and senile plaques

1991 ◽  
Vol 122 (1) ◽  
pp. 33-36 ◽  
Author(s):  
Takeo Kato ◽  
Hajime Sasaki ◽  
Tadashi Katagiri ◽  
Hideo Sasaki ◽  
Kazunori Koiwai ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Basic Fibroblast Growth Factor ◽  
Neurofibrillary Tangles ◽  
Senile Plaques ◽  
Fibroblast Growth ◽  
Alzheimer's Neurofibrillary Tangles

scholarly journals NLRP3 Inflammasome: A Starring Role in Amyloid-β- and Tau-Driven Pathological Events in Alzheimer’s Disease

2021 ◽  
pp. 1-22
Author(s):  
Mariana Van Zeller ◽  
Diogo M. Dias ◽  
Ana M. Sebastião ◽  
Cláudia A. Valente
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Glial Cells ◽  
Neurofibrillary Tangles ◽  
Nlrp3 Inflammasome ◽  
Neuronal Death ◽  
Inflammatory Responses ◽  
Senile Plaques ◽  
Amyloid Β ◽  
Wide Range

Alzheimer’s disease (AD) is the most prevalent neurodegenerative disease commonly diagnosed among the elderly population. AD is characterized by the loss of synaptic connections, neuronal death, and progressive cognitive impairment, attributed to the extracellular accumulation of senile plaques, composed by insoluble aggregates of amyloid-β (Aβ) peptides, and to the intraneuronal formation of neurofibrillary tangles shaped by hyperphosphorylated filaments of the microtubule-associated protein tau. However, evidence showed that chronic inflammatory responses, with long-lasting exacerbated release of proinflammatory cytokines by reactive glial cells, contribute to the pathophysiology of the disease. NLRP3 inflammasome (NLRP3), a cytosolic multiprotein complex sensor of a wide range of stimuli, was implicated in multiple neurological diseases, including AD. Herein, we review the most recent findings regarding the involvement of NLRP3 in the pathogenesis of AD. We address the mechanisms of NLRP3 priming and activation in glial cells by Aβ species and the potential role of neurofibrillary tangles and extracellular vesicles in disease progression. Neuronal death by NLRP3-mediated pyroptosis, driven by the interneuronal tau propagation, is also discussed. We present considerable evidence to claim that NLRP3 inhibition, is undoubtfully a potential therapeutic strategy for AD.

scholarly journals Differences in splicing defects between the grey and white matter in myotonic dystrophy type 1

2019 ◽  
Author(s):  
Masamitsu Nishi ◽  
Takashi Kimura ◽  
Mitsuru Furuta ◽  
Koichi Suenaga ◽  
Tsuyoshi Matsumura ◽  
...  
Keyword(s):  
White Matter ◽  
Myotonic Dystrophy ◽  
Cell Body ◽  
Neuronal Cell ◽  
Neuronal Cell Body ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
And Control ◽  
The Brain

AbstractMyotonic dystrophy type 1 (DM1) is a multi-system disorder caused by CTG repeats in the myotonic dystrophy protein kinase (DMPK) gene. This leads to sequestration of the splicing factor, muscleblind-like 2 (MBNL2), and aberrant splicing, mainly in the central nervous system. We investigated the splicing patterns of MBNL1/2 and genes controlled by MBNL2 in several regions of the brain and between the grey matter (GM) and white matter (WM) in DM1 patients using RT-PCR. Compared with the control, the percentage of spliced-in parameter (PSI) for most of the examined exons were significantly altered in most of the brain regions of DM1 patients, except for the cerebellum. The splicing of many genes was differently regulated between the GM and WM in both DM1 and control. The level of change in PSI between DM1 and control was higher in the GM than in the WM. The differences in alternative splicing between the GM and WM may be related to the effect of DM1 on the WM of the brain. We hypothesize that in DM1, aberrantly spliced isoforms in the neuronal cell body of the GM may not be transported to the axon. This might affect the WM as a consequence of Wallerian degeneration secondary to cell body damage. Our findings may have implications for analysis of the pathological mechanisms and exploring potential therapeutic targets.

Neurofibrillary Tangles and Senile Plaques in Aged Bears

1988 ◽  
Vol 47 (6) ◽  
pp. 629-641 ◽  
Author(s):  
Linda C. Cork ◽  
Richard E. Powers ◽  
Dennis J. Selkoe ◽  
Peter Davies ◽  
James J. Geyer ◽  
...  
Keyword(s):  
Neurofibrillary Tangles ◽  
Senile Plaques

Intraneuronal Aβ-Amyloid Precedes Development of Amyloid Plaques in Down Syndrome

2001 ◽  
Vol 125 (4) ◽  
pp. 489-492 ◽  
Author(s):  
Kymberly A. Gyure ◽  
Robert Durham ◽  
Walter F. Stewart ◽  
John E. Smialek ◽  
Juan C. Troncoso
Keyword(s):  
Down Syndrome ◽  
Alzheimer Disease ◽  
Neurofibrillary Tangles ◽  
Senile Plaques ◽  
Medical Examiner ◽  
Johns Hopkins Hospital ◽  
Sequence Of Events ◽  
Amyloid Deposits ◽  
Aβ Amyloid ◽  
Intraneuronal Aβ

Abstract Context.—Down syndrome patients who live to middle age invariably develop the neuropathologic features of Alzheimer disease, providing a unique situation in which to study the early and sequential development of these changes. Objective.—To study the development of amyloid deposits, senile plaques, astrocytic and microglial reactions, and neurofibrillary tangles in the brains of young individuals (<30 years of age) with Down syndrome. Methods.—Histologic and immunocytochemical study of a series of autopsy brains (n = 14, from subjects aged 11 months to 56 years, with 9 subjects <30 years) examined at the Office of the Chief Medical Examiner of the State of Maryland and The Johns Hopkins Hospital. Results.—The principal observations included the presence of intraneuronal Aβ immunostaining in the hippocampus and cerebral cortex of very young Down syndrome patients (preceding the extracellular deposition of Aβ) and the formation of senile plaques and neurofibrillary tangles. Conclusions.—We propose the following sequence of events in the development of neuropathologic changes of Alzheimer disease in Down syndrome: (1) intracellular accumulation of Aβ in neurons and astrocytes, (2) deposition of extracellular Aβ and formation of diffuse plaques, and (3) development of neuritic plaques and neurofibrillary tangles with activation of microglial cells.

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