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Keywords: Myelination
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Journal Articles
Dis Model Mech (2024) 17 (4): dmm050115.
Published: 9 May 2024
... muscle damage, many patients with DMD also have neurological deficits of unknown etiology. To investigate potential mechanisms for DMD neurological deficits, we assessed postnatal oligodendrogenesis and myelination in the Dmd mdx mouse model. In the ventricular-subventricular zone (V-SVZ) stem cell niche...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2021) 14 (8): dmm048925.
Published: 20 July 2021
... with the examination of cellular dynamics in vivo using animal models, such as zebrafish. Together, this will yield important insight into the cell biological mechanisms of microglial impact in the central nervous system, particularly in the development and maintenance of myelin, that will facilitate the development...
Journal Articles
Dis Model Mech (2020) 13 (7): dmm044230.
Published: 30 July 2020
...-handling deficits in MLIV occur in the lysosomes and do not involve broad iron deficiency. These data demonstrate very early deficits of oligodendrocyte maturation and critical regional differences in myelination between the forebrain and cerebellum in the mouse model of MLIV. Furthermore, they establish...
Includes: Supplementary data
Journal Articles
Dis Model Mech (2015) 8 (12): 1591–1601.
Published: 1 December 2015
... and a hypoplastic corpus callosum are the major hallmarks of MLIV brain pathology. Here, we report that loss of TRPML1 in mice results in developmental aberrations of brain myelination as a result of deficient maturation and loss of oligodendrocytes. Defective myelination is evident in Mcoln1 −/− mice at postnatal...
Includes: Supplementary data