Biomol Ther.  2017 May;25(3):231-238. 10.4062/biomolther.2016.042.

Altered Translational Control of Fragile X Mental Retardation Protein on Myelin Proteins in Neuropsychiatric Disorders

• Jeon SJ
• Ryu JH
• Bahn GH

• KMID: 2378800
• DOI: http://doi.org/10.4062/biomolther.2016.042

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.

Keyword

Myelin; Fragile X mental retardation protein; Translational control; Oligodendrocyte

MeSH Terms

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
Fragile X Mental Retardation Protein
Myelin Proteins
RNA-Binding Proteins