Oligodendrocytes in Multiple System Atrophy

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Oligodendrocytes in Multiple System Atrophy

Introduction

<table class="infobox infobox-cell">
<tr>
<th class="infobox-header" colspan="2">Oligodendrocytes in Multiple System Atrophy</th>
</tr>
<tr>
<td class="label">Taxonomy</td>
<td>ID</td>
</tr>
<tr>
<td class="label">Cell Ontology (CL)</td>
<td>[CL:4042028](https://www.ebi.ac.uk/ols4/ontologies/cl/classes/http%253A%252F%252Fpurl.obolibrary.org%252Fobo%252FCL_4042028)</td>
</tr>
<tr>
<td class="label">Database</td>
<td>ID</td>
</tr>
<tr>
<td class="label">Cell Ontology</td>
<td>[CL:4042028](https://www.ebi.ac.uk/ols4/ontologies/cl/classes/http%253A%252F%252Fpurl.obolibrary.org%252Fobo%252FCL_4042028)</td>
</tr>
<tr>
<td class="label">Region</td>
<td>MSA</td>
</tr>
<tr>
<td class="label">Striatum</td>
<td>Severe</td>
</tr>
<tr>
<td class="label">Brainstem</td>
<td>Severe</td>
</tr>
<tr>
<td class="label">Cerebellum</td>
<td>Severe</td>
</tr>
<tr>
<td class="label">Cortex</td>
<td>Mild</td>
</tr>
</table>

Oligodendrocytes In Multiple System Atrophy is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.

Overview

...

Oligodendrocytes in Multiple System Atrophy

Introduction

Overview

Mermaid diagram (expand to render)

This page provides comprehensive information about the cell type. See the content below for detailed information. [@wakabayashi1998]

Multiple system atrophy (MSA) is characterized by oligodendroglial alpha-synuclein inclusions called glial cytoplasmic inclusions (GCIs). These inclusions are the hallmark pathology and drive demyelination and neurodegeneration in this oligodendropathy. [@jellinger2014]

Multi-Taxonomy Classification

Taxonomy Database Cross-References

Morphology & Electrophysiology

Morphology: immature neuron (source: Cell Ontology)
Morphology can be inferred from Cell Ontology classification

PanglaoDB Marker Cross-References

Unknown (PanglaoDB):

External Database Links

[Cell Ontology (CL:4042028)](https://www.ebi.ac.uk/ols4/ontologies/cl/classes/http%253A%252F%252Fpurl.obolibrary.org%252Fobo%252FCL_4042028)
[OBO Foundry (CL:4042028)](http://purl.obolibrary.org/obo/CL_4042028)
[Allen Brain Cell Atlas](https://portal.brain-map.org/atlases-and-data/bkp/abc-atlas)
[CellxGene Census](https://cellxgene.cziscience.com/)
[Human Cell Atlas](https://www.humancellatlas.org/)
[PanglaoDB](https://panglaodb.se/)

Taxonomy & Classification

PanglaoDB Marker Cross-References

Unknown (PanglaoDB):

External Database Links

[Cell Ontology (CL:4042028)](https://www.ebi.ac.uk/ols4/ontologies/cl/classes/http%253A%252F%252Fpurl.obolibrary.org%252Fobo%252FCL_4042028)
[OBO Foundry (CL:4042028)](http://purl.obolibrary.org/obo/CL_4042028)
[Allen Brain Cell Atlas](https://portal.brain-map.org/atlases-and-data/bkp/abc-atlas)
[CellxGene Census](https://cellxgene.cziscience.com/)
[PanglaoDB](https://panglaodb.se/)

Glial Cytoplasmic Inclusions

Composition

Alpha-synuclein - Phosphorylated (Ser129)
Ubiquitin - Post-translational modification
P62 - Sequestosome
Tubulin - Cytoskeletal elements

Morphology

Shape: Wavy, crescent-shaped
Location: Perinuclear cytoplasm
Size: Variable (2-10 μm)
Distribution: Throughout white matter

Oligodendrocyte Dysfunction

Myelin Abnormalities

Primary oligodendropathy - Direct target
Myelin loss - Diffuse white matter changes
Layer splitting - Vacuolation
Remyelination failure - Oligodendrocyte loss

Supporting Evidence

GCIs appear before neuronal loss
Most abundant pathology
Spatial pattern matches symptoms
Animal models support oligodendrocyte origin

Affected Brain Regions

Severely Involved

Putamen - Caudate > Putamen
Pontocerebellar pathways - Brainstem
Cerebellar white matter - Ataxia correlation
Basal ganglia - Movement symptoms

Pattern Comparison

Molecular Pathogenesis

Alpha-Synuclein Aggregation

Mechanism: Unknown trigger
Propagation: Cell-to-cell spread
Strain: Distinct from PD/DLB
Oligomerization: Early toxic species

Cellular Dysfunction

Impaired myelin maintenance
Metabolic stress
Calcium dysregulation
Oxidative damage

Clinical Manifestations

Motor Symptoms (MSA-C)

Cerebellar ataxia - Gait instability
Parkinsonism - Bradykinesia, rigidity
Autonomic failure - Orthostatic hypotension

Motor Symptoms (MSA-P)

Parkinsonism predominant
Poor levodopa response
Rapid progression

Non-Motor

Sleep disorder - REM behavior disorder
Respiratory dysfunction - Stridor
Cognitive impairment - Executive dysfunction

Therapeutic Approaches

Current Management

Symptomatic - Levodopa (limited benefit)

Autonomic - Midodrine, fludrocortisone

Supportive - Physical therapy

Disease-Modifying Strategies

α-Synuclein immunotherapy - Active/passive vaccines
Aggregation inhibitors - Small molecules
Oligodendrocyte protection - Neurotrophic factors

Background

The study of Oligodendrocytes In Multiple System Atrophy has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.

Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.

External Links

[PubMed](https://pubmed.ncbi.nlm.nih.gov/) - Biomedical literature
[Alzheimer's Disease Neuroimaging Initiative](https://adni.loni.usc.edu/) - Research data
[Allen Brain Atlas](https://brain-map.org/) - Brain gene expression data

Pathway Diagram

The following diagram shows the key molecular relationships involving Oligodendrocytes in Multiple System Atrophy discovered through SciDEX knowledge graph analysis:

Mermaid diagram (expand to render)

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