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Related Concept Videos

Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Parkinson Disease ll: Pathophysiology01:24

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Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is to...
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Characterizing Histone Post-translational Modification Alterations in Yeast Neurodegenerative Proteinopathy Models
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Published on: March 24, 2019

Thinking laterally about neurodegenerative proteinopathies.

Todd E Golde1, David R Borchelt, Benoit I Giasson

  • 1Center for Translational Research in Neurodegenerative Disease, Department of Neuroscience, McKnight Brain Institute, College of Medicine, University of Florida, Gainesville, Florida 32610, USA. tgolde@mbi.ufl.edu

The Journal of Clinical Investigation
|May 3, 2013
PubMed
Summary

Neurodegenerative diseases like Alzheimer's and Parkinson's are linked to protein misfolding. Targeting downstream pathways may offer new therapeutic strategies for these proteinopathies.

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Last Updated: May 11, 2026

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Published on: July 16, 2008

Area of Science:

  • Neuroscience
  • Biochemistry
  • Pathology

Background:

  • Neurodegenerative disorders, including Alzheimer's, Parkinson's, Huntington's, and frontotemporal dementia, are often proteinopathies characterized by misfolded protein aggregation.
  • Understanding the triggers of these diseases has advanced, but translating preclinical therapies targeting these triggers into clinical success has been challenging.

Purpose of the Study:

  • To address the limited efficacy of therapies targeting initiating events in advanced-stage neurodegenerative diseases.
  • To identify common pathological processes downstream of the initial proteinopathy in multiple central nervous system (CNS) proteinopathies.
  • To explore potential strategies for developing common therapeutic targets for diverse neurodegenerative disorders.

Main Methods:

  • Review and discussion of existing literature on CNS proteinopathies.
  • Analysis of pathological processes downstream of protein aggregation triggers.
  • Identification of commonalities in degenerative cascades across multiple neurodegenerative diseases.

Main Results:

  • Therapies targeting initiating events show limited success in symptomatic patients, suggesting they may be more effective prophylactically.
  • Significant gaps exist in understanding the pathological cascades that occur after the initial protein misfolding event.
  • Common features of the downstream degenerative cascade may exist across multiple CNS proteinopathies.

Conclusions:

  • Developing therapies for advanced neurodegenerative diseases requires a deeper understanding of downstream pathology.
  • Identifying commonalities in the degenerative cascade could lead to novel, broadly applicable therapeutic targets.
  • Future research should focus on the pathological processes beyond the initial trigger to improve treatment strategies for proteinopathies.