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Nervous Tissue: Myelin

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The myelin sheath is a multilayered lipid and protein covering that insulates the axon of a neuron, enhancing the speed of nerve impulse conduction. Axons without this sheath are referred to as unmyelinated. Two types of neuroglia, Schwann cells in the peripheral nervous system (PNS) and oligodendrocytes in the central nervous system (CNS) are responsible for producing myelin sheaths.
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Myelin changes in Alexander disease.

U Gómez-Pinedo¹, M Duran-Moreno², S Sirerol-Piquer²

¹Laboratorio de Neurobiología, Servicio de Neurología, Instituto de Neurociencias, IdISSC, Hospital Clínico San Carlos, Universidad Complutense de Madrid, Madrid, España.

|March 27, 2017

Summary

This summary is machine-generated.

Alexander disease (AxD) is a leukodystrophy linked to GFAP gene mutations. This review explores alternative mechanisms beyond GFAP overproduction to explain myelin loss in AxD patients, suggesting new therapeutic targets.

Keywords:

Alexander disease Astrocitos Astrocytes Chondroitin sulfate proteoglycan-NG2 Condroitín sulfato proteoglicano-NG2 Enfermedad de Alexander Epigenetics Epigenética Glial fibrilar acidic protein Mielinización Myelination Proteína acidica fibrilar glial

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Area of Science:

Neuroscience
Genetics
Cell Biology

Background:

Alexander disease (AxD) is a fatal leukodystrophy characterized by myelin loss and Rosenthal bodies in astrocytes.
Mutations in the Glial Fibrillary Acidic Protein (GFAP) gene are the known genetic cause of AxD.
The precise molecular mechanisms linking GFAP mutations to AxD pathogenesis remain unclear.

Purpose of the Study:

To review and analyze alternative hypotheses for myelin loss in Alexander disease.
To investigate mechanisms beyond GFAP overexpression that could explain AxD pathology.
To identify potential therapeutic targets for Alexander disease.

Main Methods:

Review of current literature on Alexander disease pathogenesis.
Analysis of experimental models and genetic data.
Exploration of epigenetic, inflammatory, and cellular modification hypotheses.

Main Results:

The widespread hypothesis of GFAP gain-of-function does not fully explain myelin loss in AxD.
Alternative mechanisms, including epigenetic alterations, inflammation, NG2 (+) - GFAP (+) cells, and GFAP post-translational modifications, are proposed.
These alternative pathways offer potential explanations for the observed myelin defects.

Conclusions:

Multiple, potentially coexisting, mechanisms may underlie myelin alterations in Alexander disease.
Understanding these diverse pathways is crucial for developing effective therapies.
Targeting these novel mechanisms could lead to new therapeutic strategies for AxD.