Article
Corticospinal tract MRI hyperintensity in X-linked Charcot-Marie-Tooth Disease.
Department of Neurology, University of Ulm, Ulm, Germany.
Journal of Clinical Neuroscience (impact factor:
1.25).
07/2005;
12(5):588-9.
DOI:10.1016/j.jocn.2004.07.020
Source: PubMed
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Citations (0)
- Cited In (2)
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Article: Human oligodendrocytes express Cx31.3: function and interactions with Cx32 mutants.
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ABSTRACT: Murine oligodendrocytes express the gap junction (GJ) proteins connexin32 (Cx32), Cx47, and Cx29. CNS phenotypes in patients with X-linked Charcot-Marie-Tooth disease may be caused by dominant effects of Cx32 mutations on other connexins. Here we examined the expression of Cx31.3 (the human ortholog of murine Cx29) in human brain and its relation to the other oligodendrocyte GJ proteins Cx32 and Cx47. Furthermore, we investigated in vitro whether Cx32 mutants with CNS manifestations affect the expression and function of Cx31.3. Cx31.3 was localized mostly in the gray matter along small myelinated fibers similar to Cx29 in rodent brain and was co-expressed with Cx32 in a subset of human oligodendrocytes. In HeLa cells Cx31.3 was localized at the cell membrane and appeared to form hemichannels but no GJs. Cx32 mutants with CNS manifestations were retained intracellularly, but did not alter the cellular localization or function of co-expressed Cx31.3. Thus, Cx31.3 shares many characteristics with its ortholog Cx29. Cx32 mutants with CNS phenotypes do not affect the trafficking or function of Cx31.3, and may have other toxic effects in oligodendrocytes.Neurobiology of Disease 06/2008; 30(2):221-33. · 5.40 Impact Factor -
Article: The present and the future of neuroimaging in amyotrophic lateral sclerosis.
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ABSTRACT: In patients with ALS, conventional MR imaging is frequently noninformative, and its use has been restricted to excluding other conditions that can mimic ALS. Conversely, the extensive application of modern MR imaging-based techniques to the study of ALS has undoubtedly improved our understanding of disease pathophysiology and is likely to have a role in the identification of potential biomarkers of disease progression. This review summarizes how new MR imaging technology is changing dramatically our understanding of the factors associated with ALS evolution and highlights the reasons why it should be used more extensively in studies of disease progression, including clinical trials.American Journal of Neuroradiology 04/2010; 31(10):1769-77. · 2.93 Impact Factor
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Keywords
amyotrophic lateral sclerosis
CMTX
Connexin 32
mild subclinical CNS involvement
permanent bilateral corticospinal tract hyperintensities
visualize corticospinal tract abnormalities
X-linked hereditary demyelinating neuropathies
young male patient