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Cellular Distribution of the Fragile X Mental Retardation Protein in the Mouse Brain

Title: Cellular Distribution of the Fragile X Mental Retardation Protein in the Mouse Brain.
Name(s): Zorio, Diego A.R., author
Jackson, Christine M., author
Liu, Yong, author
Rubel, Edwin W, author
Wang, Yuan, author
Type of Resource: text
Genre: Text
Journal Article
Date Issued: 2017-03-01
Physical Form: computer
online resource
Extent: 1 online resource
Language(s): English
Abstract/Description: The fragile X mental retardation protein (FMRP) plays an important role in normal brain development. Absence of FMRP results in abnormal neuronal morphologies in a selected manner throughout the brain, leading to intellectual deficits and sensory dysfunction in the fragile X syndrome (FXS). Despite FMRP importance for proper brain function, its overall expression pattern in the mammalian brain at the resolution of individual neuronal cell groups is not known. In this study we used FMR1 knockout and isogenic wildtype mice to systematically map the distribution of FMRP expression in the entire mouse brain. Using immunocytochemistry and cellular quantification analyses, we identified a large number of prominent cell groups expressing high levels of FMRP at the subcortical levels, in particular sensory and motor neurons in the brainstem and thalamus. In contrast, many cell groups in the midbrain and hypothalamus exhibit low FMRP levels. More important, we describe differential patterns of FMRP distribution in both cortical and subcortical brain regions. Almost all major brain areas contain high and low levels of FMRP cell groups adjacent to each other or between layers of the same cortical areas. These differential patterns indicate that FMRP expression appears to be specific to individual neuronal cell groups instead of being associated with all neurons in distinct brain regions, as previously considered. Taken together, these findings support the notion of FMRP differential neuronal regulation and strongly implicate the contribution of fundamental sensory and motor processing at subcortical levels to FXS pathology.
Identifier: FSU_libsubv1_scholarship_submission_1511838919_1f7fa4f4 (IID), 10.1002/cne.24100 (DOI)
Grant Number: NIDCD DC-013074
Publication Note: The published version of the article can be found at This research was funded by a federal research grant and is made publicly accessible.
Persistent Link to This Record:
Owner Institution: FSU
Is Part Of: Journal of Comparative Neurology.
Issue: iss. 4, vol. 525

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Zorio, D. A. R., Jackson, C. M., Liu, Y., Rubel, E. W., & Wang, Y. (2017). Cellular Distribution of the Fragile X Mental Retardation Protein in the Mouse Brain. Journal Of Comparative Neurology. Retrieved from