Essential function, sophisticated regulation and pathological impact of the selective RNA-binding protein QKI in CNS myelin development

• Type of Material: Article
• Authors: Katrina Bockbrader, Emory University; Yue Feng, Emory University
• Language: English
• Date: 2008-11
• Publisher: Future Medicine
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2008 Future Medicine
• Final Published Version (URL): http://www.futuremedicine.com/doi/abs/10.2217/14796708.3.6.655
• Title of Journal or Parent Work: Future Neurology
• ISSN: 1479-6708
• Volume: 3
• Issue: 6
• Start Page: 655
• End Page: 668
• Grant/Funding Information: This work is supported by NIH grant NS39551, NMSS grant RG 4010-A-2 and NASRAD Independent Investigator Award to Yue Feng.
• Keywords: QKI; RNA-binding protein; oligodendrocytes; post-transcriptional regulation; CNS myelination; myelin disorders; schizophrenia
• Research Categories: Health Sciences, Pathology; Biology, Neuroscience; Health Sciences, Pharmacology

Abstract

The selective RNA-binding protein QKI play a key role in advancing oligodendrocyte-dependent myelination, which is essential for the function and development of the CNS. The emerging evidence that QKI abnormalities are associated with schizophrenia and may underlie myelin impairment in this devastating disease has greatly increased interest in understanding the function of QKI. Despite the discovery of the biochemical basis for QKI-RNA interaction, a comprehensive model is currently missing regarding how QKI regulates its mRNA ligands to promote normal myelinogenesis and how deficiency of the QKI pathway is involved in the pathogenesis of human diseases that affect CNS myelin. In this review, we will focus on the role of QKI in regulating distinct mRNA targets at critical developmental steps to promote oligodendrocyte differentiation and myelin formation. In addition, we will discuss molecular mechanisms that control QKI expression and activity during normal myelinogenesis as well as the pathological impact of QKI deficiency in dysmyelination mutant animals and in human myelin disorders.