Jul 30, 2010

Involvement of endoplasmic reticulum stress defined by activated unfolded protein response in multiple system atrophy

Journal of the Neurological Sciences
Kouki MakiokaKoichi Okamoto

Abstract

Multiple system atrophy (MSA) and Parkinson's disease (PD) are classified as synucleinopathies that exhibit α-synuclein deposition in the central nervous system. Recently, activation of the unfolded protein response (UPR), which is a cellular stress response triggered by endoplasmic reticulum (ER) stress, was reported in PD and involvement of ER stress was indicated for this disease. To elucidate whether ER stress is also implicated in the pathology of MSA, we performed a series of immunohistochemical studies using MSA brain sections. Here, we showed the presence of an activated UPR response in oligodendroglia of postmortem MSA brains. The UPR protein-positive structures were observed in lesions where glial cytoplasmic inclusions (GCIs) appeared and colocalized highly in cells showing oligodendrocytic characteristics in the presence of α-synuclein inclusions. The UPR protein-positive structures appeared as granular shapes that are morphologically similar to granulovacuolar degeneration (GVD) and colocalized with GVD marker proteins. Double immunohistochemistry demonstrated that some of the activated UPR protein-positive structures were localized in oligodendrocytes that contained GCIs with faint α-synuclein labeling, without ub...Continue Reading

  • References27
  • Citations21

Mentioned in this Paper

Neurofilament
Immunohistochemistry
Immunoreactivity
Formalin
Glial Fibrillary Acidic Protein
Neostriatum
Ubiquitin
Autopsy
Unfolded Protein Response
Neurons

Related Feeds

Astrocytes & Huntington’s Disease

Astrocytes are abundant within the central nervous system and their dysfunction has been thought to be an important contributor to some neurodegenerative diseases, in particular Huntington’s disease. Damage to these cells may make neurons more susceptible to degeneration. Here is the latest research on astrocytes and Huntington’s disease.

Autophagy: Cancer & Parkinson (MDS)

Autophagy leads to degradation of damaged proteins and organelles by the lysosome. Impaired autophagy has been implicated in several diseases. Here is the role of autophagy in cancer and Parkinson’s.

Autophagy & Disease

Autophagy is an important cellular process for normal physiology and both elevated and decreased levels of autophagy are associated with disease. Here is the latest research.

Alpha-Synuclein Aggregation

Alpha-synucleins are small proteins that are believed to restrict the mobility of synpatic vesicles and inhibit neurotransmitter release. Aggregation of these proteins have been linked to several types of neurodegenerative diseases including dementia with Lewy bodies and Parkinson's disease. Here is the latest research on α-synuclein aggregation.

Alpha-Synuclein Aggregation (MDS)

Alpha-synucleins are small proteins that are believed to restrict the mobility of synpatic vesicles and inhibit neurotransmitter release. Aggregation of these proteins have been linked to several types of neurodegenerative diseases including dementia with Lewy bodies and Parkinson's disease. Here is the latest research on α-synuclein aggregation.

Astrocytes

Astrocytes are glial cells that support the blood-brain barrier, facilitate neurotransmission, provide nutrients to neurons, and help repair damaged nervous tissues. Here is the latest research.

Bioinformatics in Biomedicine

Bioinformatics in biomedicine incorporates computer science, biology, chemistry, medicine, mathematics and statistics. Discover the latest research on bioinformatics in biomedicine here.

Autophagy: Cancer & Parkinson

Autophagy leads to degradation of damaged proteins and organelles by the lysosome. Impaired autophagy has been implicated in several diseases. Here is the role of autophagy in cancer and Parkinson’s.

Autophagy & Model Organisms

Autophagy is a cellular process that allows degradation by the lysosome of cytoplasmic components such as proteins or organelles. Here is the latest research on autophagy & model organisms

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.

Related Papers

Biochemical and Biophysical Research Communications
Jeroen J M HoozemansWiep Scheper
Proceedings of the National Academy of Sciences of the United States of America
Aaron D GitlerSusan Lindquist
© 2020 Meta ULC. All rights reserved