Axon-glial interaction in the CNS: what we have learned from mouse models of Pelizaeus-Merzbacher disease

2011/03/14 by Fredrik I. Gruenenfelder, Fredrik Gruenenfelder, Gemma Thomson +2 · 15 citations
Neuroscience · #Neurogenesis and neuroplasticity mechanisms #Nerve injury and regeneration #Hereditary Neurological Disorders

paper · doi:10.1111/j.1469-7580.2011.01363.x

Abstract

In the central nervous system (CNS) the majority of axons are surrounded by a myelin sheath, which is produced by oligodendrocytes. Myelin is a lipid-rich insulating material that facilitates the rapid conduction of electrical impulses along the myelinated nerve fibre. Proteolipid protein and its isoform DM20 constitute the most abundant protein component of CNS myelin. Mutations in the PLP1 gene encoding these myelin proteins cause Pelizaeus-Merzbacher disease and the related allelic disorder, spastic paraplegia type 2. Animal models of these diseases, particularly models lacking or overexpressing Plp1, have shed light on the interplay between axons and oligodendrocytes, and how one component influences the other.

Cited by

• Systematic approaches to central nervous system myelin. [europepmc]
• Cellular pathways of hereditary spastic paraplegia. [europepmc]
• Axon-glia interaction and membrane traffic in myelin formation. [europepmc]
• Early-Life Stress Perturbs Key Cellular Programs in the Developing Mouse Hippocampus. [europepmc]
• Clobetasol and Halcinonide Act as Smoothened Agonists to Promote Myelin Gene Expression and RxRγ Receptor Activation. [europepmc]
• Inherited and acquired disorders of myelin: The underlying myelin pathology. [europepmc]
• Regulation and dysregulation of axon infrastructure by myelinating glia. [europepmc]
• The Axon-Myelin Unit in Development and Degenerative Disease. [europepmc]
• Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease. [europepmc]
• Drug screening for Pelizaeus-Merzbacher disease by quantifying the total levels and membrane localization of PLP1. [europepmc]
• The wmN1 Enhancer Region of the Mouse Myelin Proteolipid Protein Gene (mPlp1) is Indispensable for Expression of an mPlp1-lacZ Transgene in Both the CNS and PNS. [europepmc]
• The Puzzle of Hereditary Spastic Paraplegia: From Epidemiology to Treatment. [europepmc]
• Aberrant Ganglioside Functions to Underpin Dysregulated Myelination, Insulin Signalling, and Cytokine Expression: Is There a Link and a Room for Therapy? [europepmc]
• PLP1 gene mutations cause spastic paraplegia type 2 in three families. [europepmc]
• From insights to innovations: evaluating preclinical paradigms in demyelinating disease therapeutics. [europepmc]

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