Myelin disorders

Before determining the chemical factors involved in myelin disorders, it is useful to first recall the morphological particularities of myelin and the Ranvier node, in CNS and PNS, as these are the major targets of demyelination. 

Myelin disorders can have a genetic, toxic, or infectious origin and some such as MS even an immunological component. For most of these diseases, there are animal models. Whatever the causes, the targets are structural or metabolic elements necessary for intact myelin. Diseases involving myelin are often related to specific myelin constituents whether common to CNS and PNS, or different. One can believe that these specialized compounds play significant roles in the pathology of myelin. Thus there are diseases or experimental models that involve only CNS or PNS, and others that involve both systems. 

Bjartmar C, Trapp BD (2001) Axonal and neuronal degeneration in multiple sclerosis mechanisms and functional consequences. Curr Opin Neurol 14 271-278 Bjartmar C, Yin X, Trapp BD (1999) Axonal pathology in myelin disorders. J Neurocytol 28 383-395... 

Meyer zu Horste G, Pmkop T, Nave KA, Sereda MW (2006) Myelin disorders causes and perspectives of Charcot-Marie-Tooth neuropathy. J Mol Neurosd 28 77-88... 

Stallberg-Stenhagen S, Svennerhohn L. Fatty acid composition of human brain sphingomyelins. Normal variation with age and changes during myelin disorders. J. Lipid Res. 1965 6 146-155. 

Death of OL and subsequent myelin loss has been reported in a variety of myelin disorders including, multiple sclerosis (MS), X-adrenoleukodystrophy (X-ALD), adrenomyeloneu-ropathy (AMN), vascular dementia, periventricular leukomalacia (PVL), hypoxia, and ischemia. Several factors that might be associated with OL death in these pathophysiological concU-dons are discussed below. 

The influence of the immune system on disease progression in genetic myelin disorders. 

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