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

doi:10.1083/jcb.152.1.75

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 Pellizzoni^a, Bernard Charroux^a, Juri Rappsilber^b, Matthias Mann^b, and Gideon Dreyfuss^a

^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 productof the spinal muscular atrophy (SMA) disease gene, plays a rolein 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–associatedprotein. RHA is a DEAH box RNA helicase which binds RNA polymeraseII (pol II) and reportedly functions in transcription. SMN interactswith RHA in vitro, and this interaction is impaired in mutantSMNs found in SMA patients. Coimmunoprecipitation demonstratedthat the SMN complex is associated with pol II, snRNPs, andRHA in vivo. In vitro experiments suggest that RHA mediatesthe association of SMN with the COOH-terminal domain of polII. Moreover, transfection of cells with a dominant negativemutant of SMN, SMN ${Delta}$ N27, causes accumulation of pol II, snRNPs,and RHA in nuclear structures that contain the known markersof gems and coiled bodies, and inhibits RNA pol I and pol IItranscription in vivo. These findings indicate a functionalas well as physical association of the SMN complex with polII and suggest a role for the SMN complex in the assembly ofthe pol II transcription/processing machinery.

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

Abbreviations used in this paper: BRCA1, breast cancer gene1; Bru, bromouridine; CBP, cAMP response element binding protein;CTD, COOH-terminal domain; G/CB, nuclear accumulation containinggem- and coiled (cajal) body-specific marker; GST, glutathioneS-transferase; hnRNP, heterogeneous nuclear RNP; IGC, interchromatingranule 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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