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Published online 8 January 2001. doi:10.1083/jcb.152.1.75
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© The Rockefeller University Press, 0021-9525/2001//75 $5.00
The Journal of Cell Biology, Volume 152, Number 1, , 2001 75-86


Original Article

A Functional Interaction between the Survival Motor Neuron Complex and RNA Polymerase II



Livio Pellizzonia, Bernard Charrouxa, Juri Rappsilberb, Matthias Mannb, and Gideon Dreyfussa

a Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104
b Protein Interaction Laboratory, University of Southern Denmark, DK-5230 Odense M, Denmark
Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, 415 Curie Blvd., Clinical Research Bldg., Rm. 328, Philadelphia, PA 19104-6148.(215) 573-2000(215) 898-0398

gdreyfuss{at}hhmi.upenn.edu

The survival motor neuron (SMN) protein, the protein product of the spinal muscular atrophy (SMA) disease gene, plays a role in the assembly and regeneration of small nuclear ribonucleoproteins (snRNPs) and spliceosomes. By nanoelectrospray mass spectrometry, we identified RNA helicase A (RHA) as an SMN complex–associated protein. RHA is a DEAH box RNA helicase which binds RNA polymerase II (pol II) and reportedly functions in transcription. SMN interacts with RHA in vitro, and this interaction is impaired in mutant SMNs found in SMA patients. Coimmunoprecipitation demonstrated that the SMN complex is associated with pol II, snRNPs, and RHA in vivo. In vitro experiments suggest that RHA mediates the association of SMN with the COOH-terminal domain of pol II. Moreover, transfection of cells with a dominant negative mutant of SMN, SMN{Delta}N27, causes accumulation of pol II, snRNPs, and RHA in nuclear structures that contain the known markers of gems and coiled bodies, and inhibits RNA pol I and pol II transcription in vivo. These findings indicate a functional as well as physical association of the SMN complex with pol II and suggest a role for the SMN complex in the assembly of the pol II transcription/processing machinery.

Key Words: survival motor neuron • RNA helicase A • RNA polymerase II • nuclear bodies • transcriptosome



© 2001 The Rockefeller University Press

Abbreviations used in this paper: BRCA1, breast cancer gene 1; Bru, bromouridine; CBP, cAMP response element binding protein; CTD, COOH-terminal domain; G/CB, nuclear accumulation containing gem- and coiled (cajal) body-specific marker; GST, glutathione S-transferase; hnRNP, heterogeneous nuclear RNP; IGC, interchromatin granule cluster; pol, polymerase; RHA, RNA helicase A; RNP, ribonucleoprotein; Sm, Smith antigen; snRNP, small nuclear RNP; SMA, spinal muscular atrophy; SMN, survival motor neuron; SR, serine- and arginine-rich; TBP, TATA box binding protein.



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