The human dystrophin gene requires 16 hours to be transcribed and is cotranscriptionally spliced

Nature Genetics

Volumn 9, Issue 2, 1995, Pages 184-190

  Tennyson, Christine N ^a,b   Klamut, Henry J ^a   Worton, Ronald G ^a,b  

^a HOSPITAL FOR SICK CHILDREN   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

DNA; DYSTROPHIN; RNA;

ARTICLE; CELL CULTURE; CONTROLLED STUDY; DENSITOMETRY; FETUS; GENE; GENETIC TRANSCRIPTION; HUMAN; HUMAN CELL; MUSCLE CELL; POLYACRYLAMIDE GEL ELECTROPHORESIS; POLYMERASE CHAIN REACTION; PRIORITY JOURNAL; RNA SPLICING;

ANIMAL; BASE SEQUENCE; CELL DIFFERENTIATION; DNA, RECOMBINANT; DYSTROPHIN; EXONS; GENE EXPRESSION; HUMAN; MOLECULAR SEQUENCE DATA; MUSCLES; POLYMERASE CHAIN REACTION; SUPPORT, NON-U.S. GOV'T; TIME FACTORS; TRANSCRIPTION, GENETIC;

EID: 0028837312     PISSN: 10614036     EISSN: 15461718     Source Type: Journal    
DOI: 10.1038/ng0295-184     Document Type: Article

References (43)

1. Den Dunnen, J.Y. et al. Topography of the Duchenne muscular dystrophy (DMD) gene: FIGE and cDNA analysis of 194 cases reveals 115 deletions and 13 duplications. Am. J. hum. Genet. 45, 835-847 (1989).
2. Coffey, A.J. et al. Construction of a 2.6-Mb contig In yeast artificial chromosomes spanning the human dystrophin gene using an STS-based approach. Genomics 12, 474-484 (1992).
3. Monaco, A.P., Walker, A.P., Millwood, I., Larin, Z. & Lehrach, H. A yeast artificial chromosome contig containing the complete Duchenne muscular dystrophy gene. Genomics 12, 465-473 (1992).
4. Roberts, R.G., Coffey, A.J., Bobrow, M. & Bentley, D.R. Determination of the exon structure of the distal portion of the dystrophin gene by vectorette PCR. Genomics 13, 942-950 (1992).
5. Koenig, M., Monaco, A.P. & Kunkel, L.M. The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell 53, 219-228 (1988).
6. Ucker, D.S. & Yamamoto, K.R. Early events in the stimulation of mammary tumor virus RNA synthesis by glucocorticoids. J. biol. Chem. 259, 7416-7420 (1984).
7. Sehgal, P.B., Derman, E., Molloy, G.R., Tamm, I. & Darnell, J.E. 5-6-Dichloro-1-B-D-ribofuranosylbenzimide inhibits initiation of nuclear heterogeneous RNA chains in Hala cells. Science 194, 431-433 (1976).
8. Lederfein, D., Yaffe, D. & Nudel, U. A housekeeping type promoter, located in the 3' region of the Duchenne muscular dystrophy gene, controls the expression of Dp71, a major product of the gene. Hum. molec. Genet. 2, 1883-1888 (1993).
9. Gorecki, D.C. et al. Expression of four alternative dystrophin transcripts in brain regions regulated by different promoters. Hum. molec. Genet. 1, 505-510 (1992).
10. Byers, T.J., Lidov, H.G.W. & Kunkel, L.M. An alternative dystrophin transcript specific to peripheral nerve. Nature Genet. 4, 77-81 (1993).
11. Chelly, J., Montarras, D., Pinset, C., Berwald-Netter, Y. & Kaplan, J.C. Quantitative estimation of minor mRNAs by cDNA-polymerase chain reaction: application to dystrophin mRNA in cultured myogenic and brain cells. Eur. J. Biochem. 187, 691-698 (1990).
12. Nudel, U., Robzyk, K. & Yaffe, D. Expression of the putative Duchenne muscular dystrophy gene in differentiated myogenic cell cultures and in the brain. Nature 331, 635-638 (1988).
13. Lev, A.A., Feener, C.C., Kunkel, L.M. & Brown, R.H. Expression of the Duchenne's muscular dystrophy gene in cultured muscle cells. J. biol. Chem. 262, 15817-15820 (1987).
14. Klamut, H.J., Gangopadhyay, S.B., Worton, R.G. & Ray, P.N. Molecular and functional analysis of the muscle-specific promoter region of the Duchenne muscular dystrophy gene. Molec. cell. Biol. 10, 193-205 (1990).
15. Krause, M. & Hirsh, D. A trans-spliced leader sequence on actin mRNA in C. elegans. Cell 49, 753-761 (1987).
16. Feener, C.A., Koenig, M. & Kunkel, L.M. Alternative splicing of human dystrophin mRNA generates isoforms at the carboxy terminus. Nature 338, 509-511 (1989).
17. Bies, R.D. et al. Human and murine dystrophin mRNA transcripts are differentially expressed during skeletal muscle, heart & brain development. Nucl. Acids Res. 20, 1725-1731 (1992).
18. Bar, S. et al. A novel product of the Duchenne muscular dystrophy gene which greatly differs from known isoforms in Its structure and tissue distribution. Biochem. J. 272, 557-560 (1990).
19. Byers, T.J., Lidov, H.G.W. & Kunkel, L.M. An alternative dystrophin transcript specific to peripheral nerve. Nature Genet. 4, 77-81 (1993).
20. Hugnot, J.P. et al. Distal transcript of the dystrophin gene initiated from an alternative first exon and encoding a 75-kDa protein widely distributed in non-muscle tissues. Proc. natn. Acad. Sci. U.S.A. 89, 7506-7510 (1992).
21. Lederfein, D. et al. A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other non-muscle tissues. Proc. natn. Acad. Sci. U.S.A. 89, 5346-5350 (1992).
22. Thummel, C.S., Burtis, K.C., Hogness, D.S. Spatial and temporal patterns of E74 transcription during drosophila development. Cell 61, 101-111 (1990).
23. Karim, F.D. & Thummel, C.S. Ecdysone coordinates the timing and amounts of E74A and E74B trancription in Drosophlia. Genes Dev. 5, 1067-1079 (1991).
24. Shermoen, A.W. & O'Farrell, P.H. Progression of the cell cycle through mitosis leads to abortion of nascent transcripts. Cell 67, 303-310 (1991).
25. Blau, H.M., Webster, C., Pavlath, G.K. & Chiu, C. Evidence for defective myoblasts in Duchenne muscular dystrophy. Adv Exp. Medicine Bio. 182, 85-110 (1985).
26. Nevins, J.R. & Darnell, J.E. Steps in the processing of Ad2 mRNA Poly (A)+ nuclear sequences are conserved and poly (A) addition precedes splicing. Cell 15, 1477-1493 (1978).
27. Ford, J.P. & Hus, M. Transcription pattern of in vivo-labeled late simian virus 40 RNA: equimolar transcription beyond the mRNA 3' terminus. J. Virol. 28, 795-801 (1978).
28. Weber, J., Blanchard, J.M., Binsberg, H., Darnell, J.E. Order of polyadenylic acid addition and splicing events In early adenovirus mRNA formation. J. Virol. 33, 286-291 (1980).
29. Galli, G., Guise, J., Tucker, P.W., Nevins, J.R. Poly(A) site choice rather than splice site choice governs the regulated production of IgM heavy-chain RNAs. Proc. natn. Acad. Sci. U.S.A. 85, 2439-2443 (1988).
30. Beyer, A.L. & Osheim, Y.N. Splice site selection, rate of splicing & alternative splicing on nascent transcripts. Genes Dev. 2, 754-765 (1988).
31. LeMaire, M.F. & Thummel, C.S. Splicing precedes polyadenylatlon during drosophila E74A transcription. Molec. cell Biol. 10, 6059-6063 (1990).
32. Bauren, G. & Wieslander, L. Splicing of Balbiani ring 1 gene pre-mRNA occurs simultaneously with transcription. Cell 76, 183-192 (1994).
33. Zeevi, M., Nevins, J.R. & Darnell, J.E. Jr. Nuclear RNA is spliced in the absence of poly (A) addition. Cell 26, 39-46 (1981).
34. Abei, M. & Weissmann, C. Precision and orderliness in splicing. Trend Genet. 3, 102-107 (1987).
35. Reed, R. & Maniatis, T. A role for exon sequences and splice-site proximity in splice-site selection. Cell 46, 681-690 (1986).
36. Solnick, D. Alternative splicing caused by RNA secondary structure. Cell 43, 667-676 (1985).
37. Hedley, M.L. & Maniatis, T. Sex-specific splicing and polyadenylatlon of dsx pre-mRNA requires a sequence that binds specifically to tra-2 protein in vitro. Cell 65, 579-586 (1991).
38. Lee, H., Kraus, K.W., Wolfner, M.F. & Lis, J.T. DNA sequence requirements for generating paused polymerase at the start of hsp70.Genes Dev. 6, 284-295 (1992).
39. London, L., Keene, R.G. & Landick, R. Analysis of premature termination in c-myc during transcription by RNA polymerase II in a Hela nuclear extract. Molec. cell. Biol. 11, 4599-4615 (1991).
40. Spencer, C.A. & Groudine, M. Transcription elongation and eukaryotic gene regulation. Oncogene 5, 777-785 (1990).
41. MacDonald, R.J., Swift, G.H., Przybyla, A.E. & Chirgwin, J.M. Isolation of RNA using guanidinium salts. Meth. Enzymol. 152, 219-227 (1987).
42. Maniatis, T., Fritsch, E.F. & Sambrook, J. Molecular cloning: a laboratory manual. (Cold Spring Harbor Laboratory, New York, 1989).
43. Wang, M., Doyle, M.V. & Mark, D.F. Quantitation of mRNA by the polymerase chain reaction. Proc. natn. Acad. Scl. U.S.A. 86, 9717-9721 (1989).