The quakingviable mutation affects qkl mRNA expression specifically in myelin-producing cells of the nervous system

Nucleic Acids Research

Volumn 31, Issue 15, 2003, Pages 4616-4624

  Lu, Zifan ^a   Zhang, Youyi ^a   Ku, Li ^a   Wang, Houping ^a   Ahmadian, Amir ^a   Feng, Yue ^a  

^a EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ISOPROTEIN; MESSENGER RNA; MYELIN; RNA BINDING PROTEIN; MYELIN BASIC PROTEIN; QK PROTEIN, MOUSE;

ANIMAL CELL; ARTICLE; CARBOXY TERMINAL SEQUENCE; CENTRAL NERVOUS SYSTEM; CONTROLLED STUDY; EXON; GENE DELETION; GENE EXPRESSION; GENE LOCATION; GENE MUTATION; GENETIC TRANSCRIPTION; HEART; IMMUNOHISTOCHEMISTRY; MACROGLIA; MOUSE; MOUSE MUTANT; MYELINATION; NONHUMAN; PERIPHERAL NERVOUS SYSTEM; PRIORITY JOURNAL; RNA PROCESSING; RNA SPLICING; TESTIS; TISSUE DISTRIBUTION; ANIMAL; ANTIBODY SPECIFICITY; BRAIN; CELL CULTURE; CYTOLOGY; GENE EXPRESSION REGULATION; GENETICS; GLIA; METABOLISM; MUTATION; MYELIN SHEATH; NERVOUS SYSTEM; PROTEIN SYNTHESIS;

ANIMALIA;

ANIMALS; BRAIN; CELLS, CULTURED; CENTRAL NERVOUS SYSTEM; GENE EXPRESSION REGULATION; MICE; MICE, QUAKING; MUTATION; MYELIN BASIC PROTEINS; MYELIN SHEATH; NERVOUS SYSTEM; NEUROGLIA; ORGAN SPECIFICITY; PERIPHERAL NERVOUS SYSTEM; PROTEIN BIOSYNTHESIS; PROTEIN ISOFORMS; RNA, MESSENGER; RNA-BINDING PROTEINS;

EID: 0042662877     PISSN: 03051048     EISSN: None     Source Type: Journal    
DOI: 10.1093/nar/gkg635     Document Type: Article

References (22)

1. Hardy, R.J. (1998) Molecular defects in the dysmyelinating mutant quaking. J. Neurosci. Res., 51, 417-422.
2. Campagnoni, A.T. and Macklin, W.B. (1988) Cellular and molecular aspects of myelin protein gene expression. Mol. Neurobiol., 2, 41-89.
3. Sidman, S., Dickie, M. and Appel, S. (1964) Mutant mice (Quaking and Jimpy) with deficient myelination in the central nervous system. Science, 144, 309-311.
4. Hogan, E.L. and Greenfield, S. (1984) Animal Models of Genetic Disorders of Myelin. Plenum Press, New York, NY.
5. Billings-Gagliardi, S., Adcock, L.H. and Wolf, M.K. (1980) Hypomyelinated mutant mice: description of jpmsd and comparison with jp and qk on their present genetic backgrounds. Brain Res., 194, 325-338.
6. Ebersole, T.A., Chen, Q., Justice, M.J. and Artzt, K. (1996) The quaking gene product necessary in embryogenesis and myelination combines features of RNA binding and signal transduction proteins. Nature Genet., 12, 260-265.
7. Kondo, T., Furuta, T., Mitsunaga, K., Ebersole, T.A., Shichiri, M., Wu, J., Artzt, K., Yamamura, K. and Abe, K. (1999) Genomic organization and expression analysis of the mouse qkI locus. Mamm. Genome, 10, 662-669.
8. Hardy, R.J., Loushin, C.L., Friedrich, V.L., Jr, Chen, Q., Ebersole, T.A., Lazzarini, R.A. and Artzt, K. (1996) Neural cell type-specific expression of QKI proteins is altered in quakingviable mutant mice. J. Neurosci., 16, 7941-7949.
9. Vernet, C. and Artzt, K. (1997) STAR, a gene family involved in signal transduction and activation of RNA. Trends Genet., 13, 479-484.
10. Li, Z., Zhang, Y., Li, D. and Feng, Y. (2000) Destabilization and mislocalization of myelin basic protein mRNAs in quaking dysmyelination lacking the QKI RNA-binding proteins. J. Neurosci., 20, 4944-4953.
11. Wu, J.I., Reed, R.B., Grabowski, P.J. and Artzt, K. (2002) Function of quaking in myelination: regulation of alternative splicing. Proc. Natl Acad. Sci. USA, 99, 4233-4238.
12. Larocque, D., Pilotte, J., Chen, T., Cloutier, F., Massie, B., Pedraza, L., Couture, R., Lasko, P., Almazan, G. and Richard, S. (2002) Nuclear retention of MBP mRNAs in the quaking viable mice. Neuron, 36, 815-829.
13. Wu, J., Zhou, L., Tonissen, K., Tee, R. and Artzt, K. (1999) The quaking I-5 protein (QKI-5) has a novel nuclear localization signal and shuttles between the nucleus and the cytoplasm. J. Biol. Chem., 274, 29202-29210.
14. Zhang, Y. and Feng, Y. (2001) Distinct molecular mechanisms lead to diminished myelin basic protein and 2′, 3′-cyclic nucleotide 3′-phosphodiesterase in qk(v) dysmyelination. J. Neurochem., 77, 165-172.
15. Knapp, P.E., Bartlett, W.P. and Skoff, R.P. (1987) Cultured oligodendrocytes mimic in vivo phenotypic characteristics: cell shape, expression of myelin-specific antigens and membrane production. Dev. Biol., 120, 356-365.
16. Feng, Y., Gutekunst, C.A., Eberhart, D.E., Yi, H., Warren, S.T. and Hersch, S.M. (1997) Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes. J. Neurosci., 17, 1539-1547.
17. Feng, Y., Absher, D., Eberhart, D.E., Brown, V., Malter, H.E. and Warren, S.T. (1997) FMRP associates with polyribosomes as an mRNP and the I304N mutation of severe fragile X syndrome abolishes this association. Mol. Cell, 1, 109-118.
18. Zhang, Y., Lu, Z., Ku, L., Chen, Y., Wang, H. and Feng, Y. (2003) Tyrosine-phosphorylation of QKI mediates developmental signals to regulate mRNA metabolism. EMBO J., 22, 1801-1810.
19. Ashley, C.T., Jr, Wilkinson, K.D., Reines, D. and Warren, S.T. (1993). FMR1 protein: conserved RNP family domains and selective RNA binding. Science, 262, 563-566.
20. Gow, A., Southwood, C.M. and Lazzarini, R.A. (1998) Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J. Cell Biol., 140, 925-934.
21. Sperber, B.R., Boyle-Walsh, E.A., Engleka, M.J., Gadue, P., Peterson, A.C., Stein, P.L., Scherer, S.S. and McMorris, F.A. (2001) A unique role for Fyn in CNS myelination. J. Neurosci., 21, 2039-2047.
22. Fruttiger, M., Karlsson, L., Hall, A.C., Abramsson, A., Calver, A.R., Bostrom, H., Willetts, K., Bertold, C.H., Heath, J.K., Betsholtz, C. and Richardson, W.D. (1999) Defective oligodendrocyte development and severe hypomyelination in PDGF-A knockout mice. Development, 126, 457-467.