Altered Translational Control of Fragile X Mental Retardation Protein on Myelin Proteins in Neuropsychiatric Disorders.
10.4062/biomolther.2016.042
- Author:
Se Jin JEON
1
;
Jong Hoon RYU
;
Geon Ho BAHN
Author Information
1. Department of Life and Nanopharmaceutical Science, College of Pharmacy, Republic of Korea. jhryu63@khu.ac.kr
- Publication Type:Review
- Keywords:
Myelin;
Fragile X mental retardation protein;
Translational control;
Oligodendrocyte
- MeSH:
Axons;
Cell Body;
Central Nervous System;
Fragile X Mental Retardation Protein*;
Fragile X Syndrome;
Gene Expression;
Myelin Proteins*;
Myelin Sheath*;
Nervous System;
Neuroglia;
Neurons;
Oligodendroglia;
Risk Factors;
RNA-Binding Proteins;
White Matter
- From:Biomolecules & Therapeutics
2017;25(3):231-238
- CountryRepublic of Korea
- Language:English
-
Abstract:
Myelin is a specialized structure of the nervous system that both enhances electrical conductance and insulates neurons from external risk factors. In the central nervous system, polarized oligodendrocytes form myelin by wrapping processes in a spiral pattern around neuronal axons through myelin-related gene regulation. Since these events occur at a distance from the cell body, post-transcriptional control of gene expression has strategic advantage to fine-tune the overall regulation of protein contents in situ. Therefore, many research interests have been focused to identify RNA binding proteins and their regulatory mechanism in myelinating compartments. Fragile X mental retardation protein (FMRP) is one such RNA binding protein, regulating its target expression by translational control. Although the majority of works on FMRP have been performed in neurons, it is also found in the developing or mature glial cells including oligodendrocytes, where its function is not well understood. Here, we will review evidences suggesting abnormal translational regulation of myelin proteins with accompanying white matter problem and neurological deficits in fragile X syndrome, which can have wider mechanistic and pathological implication in many other neurological and psychiatric disorders.
