title: QUAKING KH domain proteins as regulators of glial cell fate and myelination.
abstract: The quaking viable (qk(v)) mice have attracted attention because of their characteristic tremor caused by their dysmyelination. In the central nervous system, qk(v) mice fail to develop mature myelinating oligodendrocytes and display uncompacted myelin. The genetic defect in the qk(v) mice prevents the proper expression of alternatively spliced KH-type QKI RNA binding proteins. Thus qk(v) mice provide a unique animal model linking RNA binding proteins to defects in oligodendrocyte cell fate and myelination. The fact that QKI proteins are modified post-translationally makes them Signal Transduction Activiators of RNA (STAR) proteins. We have used a gain-of-function approach with the ectopic expression of the separate QKI isoforms using adenoviruses and retroviruses to determine their separate roles in cell fate and myelination. Herein, we discuss the recent advances in characterizing the QKI KH-type proteins as glial cell fate and myelin egulators.
authors.affiliation: Terry Fox Molecular Oncology Group and the Bloomfield Center for Research on Aging, Lady Davis Institute for Medical Research, Sir Mortimer B. Davis Jewish General Hospital, Departments of Oncology and Medicine, McGill University, Montréal, Québec, Canada.
authors.forename: Daniel
authors.initials: D
authors.lastname: Larocque
authors.forename: Stéphane
authors.initials: S
authors.lastname: Richard
mesh_terms: D000818:Animals; D002454:Cell Differentiation; D050760:Cyclin-Dependent Kinase Inhibitor p27 / Q000502:physiology; D005328:Fetal Viability; D051379:Mice; D008821:Mice, Quaking; D008954:Models, Biological; D004676:Myelin Basic Protein / Q000378:metabolism; D009186:Myelin Sheath / Q000502:physiology*; D009457:Neuroglia / Q000166:cytology*; D009836:Oligodendroglia / Q000166:cytology; D017434:Protein Structure, Tertiary; D016601:RNA-Binding Proteins / Q000378:metabolism / Q000502:physiology*
chemical_list: D004676:Myelin Basic Protein; C490832:QKI protein, human; C098217:Qk protein, mouse; D016601:RNA-Binding Proteins; D050760:Cyclin-Dependent Kinase Inhibitor p27