Browsing by Subject "Oligodendrocyte"

Now showing 1 - 3 of 3
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    Growth factors and remyelination in the CNS
    (Murcia : F. Hernández, 1997) Woodruff, R.H.; Franklin, R.J.M.
    It is now well established that there is an inherent capacity within the central nervous system (CNS) to remyelinate areas of white matter that have undergone demyelination. However this repair process is not universally consistent or sustained, and persistent demyelination occurs in a number of situations, most notably in the chronic multiple sclerosis (MS) plaque. Thus there is a need to investigate ways in which myelin deficits within the CNS rnay be restored. One approach to this problem is to investigate ways in which the inherent remyelinating capacity of the CNS rnay be stimulated to remyelinate areas of long-term demyelination. The expression of growth factors, which are known to be involved in developmental myelinogenesis, in areas of demyelination strongly suggests that they are involved in spontaneous remyelination. Therefore delivery of exogenous growth factors into areas of persistent demyelination is a potential therapeutic strategy for stimulating remyelination. This review will discuss the evidence that growth factors rnay have a role in promoting CNS remyelination by enhancing the survival and stimulating the proliferation and recruitment of remyelinating oligodendrocytes.
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    Molecular mechanisms of gap junction mutations in myelinating cells
    (Murcia : F. Hernández, 2010) Sargiannidou, Irene; Markoullis, Kyriaki; Kleopa, Kleopas A.
    There is an emerging group of neurological disorders that result from genetic mutations affecting gap junction proteins in myelinating cells. The X-linked form of Charcot Marie Tooth disease (CMT1X) is caused by numerous mutations in the GJB1 gene encoding the gap junction protein connexin32 (Cx32), which is expressed in both Schwann cells in the PNS and oligodendrocytes in the CNS. Patients with CMT1X present mainly with a progressive peripheral neuropathy, showing mixed axonal and demyelinating features. In many cases there is also clinical or subclinical involvement of the CNS with acute or chronic phenotypes of encephalopathy. Furthermore, mutations in the GJA12/GJC2 gene encoding the gap junction protein Cx47, which is expressed in oligodendrocytes, have been identified in families with progressive leukodystrophy, known as Pelizaeus-Merzbacher-like disease, as well as in patients with hereditary spastic paraplegia. Recent studies have provided insights into the pattern of gap junction protein expression and function in CNS and PNS myelinating cells. Furthermore, in vitro and in vivo disease models have clarified some of the molecular and cellular mechanisms underlying these disorders. Here we provide an overview of the clinical, genetic, and neurobiological aspects of gap junction disorders affecting the nervous system.
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    Multiple sclerosis - remyelination failure as a cause of disease progression
    (F. Hernandez y JuanF. Madrid. Universidad de Murcia. Departamento de Biología Celular e Histología., 2012) Hagemeier, Karin; Brück, Wolfgang; Kuhlmann, Tanja
    Multiple sclerosis (MS) is the most frequent demyelinating disease of the central nervous system (CNS) that affects worldwide about 2.5 million people. The morphological correlates of the disease are multiple lesions in brain and spinal cord which are characterized by demyelination, inflammation, gliosis and axonal damage. The underlying cause for the permanent neurological deficits in MS patients is axonal loss. Demyelinated axons are prone to damage due to the lack of trophic support by myelin sheaths and oligodendrocytes, as well as the increased vulnerability to immune mediated attacks. Remyelination occurs, but especially in chronic lesions is frequently limited to a small rim at the lesion border. Current treatment strategies are based on anti-inflammatory or immunomodulatory drugs and have the potential to reduce the numbers of newly evolving lesions, although as yet no treatment strategy exists to influence or prevent the progressive disease phase. Therefore, the development of neuroprotective treatment options, such as the promotion of endogenous remyelination is an attractive strategy. A prerequisite for the development of such new treatments is the understanding of the mechanisms leading to remyelination and the reasons for insufficient endogenous repair in chronic MS. This review will therefore provide an overview of the current concepts regarding remyelination in the rodent and human CNS. We will also summarize a selected number of inhibitory pathways and non-disease related factors which may contribute to remyelination failure in chronic MS.