Oligodendrocytes in Multiple System Atrophy

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">Feature</td>
<td>Description</td>
</tr>
<tr>
<td class="label">GCI burden</td>
<td>Correlates with disease severity</td>
</tr>
<tr>
<td class="label">Neuronal loss</td>
<td>Associated with GCI density</td>
</tr>
<tr>
<td class="label">Axonal degeneration</td>
<td>Secondary to demyelination</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

<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">Feature</td>
<td>Description</td>
</tr>
<tr>
<td class="label">GCI burden</td>
<td>Correlates with disease severity</td>
</tr>
<tr>
<td class="label">Neuronal loss</td>
<td>Associated with GCI density</td>
</tr>
<tr>
<td class="label">Axonal degeneration</td>
<td>Secondary to demyelination</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

Multiple System Atrophy (MSA) is a progressive neurodegenerative disorder characterized by autonomic failure, parkinsonism, and cerebellar ataxia. A defining pathological feature is the presence of glial cytoplasmic inclusions (GCIs) in oligodendrocytes, making it a primary oligodendrogliopathy. [@wenning2022]

<!-- taxonomy-enrichment --> [@koga2021]

<!-- multi-taxonomy-enrichment -->

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 (GCIs)

Structure and Composition

• Main component: [Alpha-synuclein](/proteins/alpha-synuclein) (α-syn)
• Filamentous nature: Made of 10-12nm filaments
• Distribution: Throughout white matter

Formation Mechanisms

• α-Synuclein misfolding: From neuronal to glial transmission
• Oligodendroglial vulnerability: Low proteasomal activity
• Myelin stress: Metabolic vulnerability

Oligodendrocyte Dysfunction

Myelin Abnormalities

• Primary degeneration: Loss of myelin-producing cells
• Secondary demyelination: Due to oligodendrocyte death
• White matter lesions: On MRI

Cellular Changes

Regional Vulnerability

Striatonigral Degeneration (MSA-P)

• Putamen: Severe neuronal loss
• Globus pallidus: Iron deposition
• Substantia nigra: Dopaminergic neuron loss

Olivopontocerebellar Atrophy (MSA-C)

• Inferior olivary nucleus: Neuronal loss
• Pontine nuclei: Demyelination
• Cerebellar white matter: GCI accumulation

Molecular Mechanisms

Alpha-Synuclein Pathology

• Phosphorylation: Ser129 phosphorylation in GCIs
• Aggregation: Toxic oligomer formation
• Transmission: Neuron-to-glia and glia-to-neuron

Cellular Stress Pathways

• ER stress: CHOP activation
• Mitochondrial dysfunction: Complex I deficiency
• Inflammation: Microglial activation

Therapeutic Implications

Current Challenges

• No disease-modifying therapy: Limited treatment options
• Rapid progression: Median survival 6-9 years

Emerging Approaches

• α-Synuclein targeting: Immunotherapies
• Neuroprotective agents: CoQ10, mesenchymal stem cells
• Myelin repair: Oligodendrocyte precursor activation

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.

See Also

• [Parkinson's Disease](/diseases/parkinsons-disease)
• [Alpha-Synuclein](/mechanisms/alpha-synuclein)
• [/mechanisms/app-processing](/mechanisms/app-processing)
• [/mechanisms/amyloid-aggregation](/mechanisms/amyloid-aggregation)
• [/mechanisms/microglia-neuroinflammation](/mechanisms/microglia-neuroinflammation)

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: