Title: Transcriptional upregulation of myelin components in spontaneous myelin basic protein-deficient mice
Authors: Staats, Kim A
Pombal, Diana
Schönefeldt, Susann
Van Helleputte, Lawrence
Maurin, Hervé
Dresselaers, Tom
Govaerts, Kristof
Himmelreich, Uwe
Van Leuven, Fred
Van Den Bosch, Ludo
Dooley, James
Humblet-Baron, Stephanie #
Liston, Adrian # ×
Issue Date: May-2015
Publisher: Elsevier/North Holland
Series Title: Brain Research vol:1606 pages:125-32
Article number: S0006-8993(15)00121-3
Abstract: Myelin is essential for efficient signal transduction in the nervous system comprising of multiple proteins. The intricacies of the regulation of the formation of myelin, and its components, are not fully understood. Here, we describe the characterization of a novel myelin basic protein (Mbp) mutant mouse, mbp(jive), which spontaneously occurred in our mouse colony. These mice displayed the onset of a shaking gait before 3 weeks of age and seizure onset before 2 months of age. Due to a progressive increase of seizure intensity, mbp(jive) mice experienced premature lethality at around 3 months of age. Mbp mRNA transcript or protein was undetectable and, accordingly, genetic analysis demonstrated a homozygous loss of exons 3 to 6 of Mbp. Peripheral nerve conductance was mostly unimpaired. Additionally, we observed grave structural changes in white matter predominant structures were detected by T1, T2 and diffusion weighted magnetic resonance imaging. We additionally observed that Mbp-deficiency results in an upregulation of Qkl, Mag and Cnp, suggestive of a regulatory feedback mechanism whereby compensatory increases in Qkl have downstream effects on Mag and Cnp. Further research will clarify the role and specifications of this myelin feedback loop, as well as determine its potential role in therapeutic strategies for demyelinating disorders.
ISSN: 0006-8993
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Laboratory of Genetics of Autoimmunity
Laboratory for Neurobiology
Biomedical MRI
× corresponding author
# (joint) last author

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