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Originally published In Press as doi:10.1074/jbc.M208287200 on November 7, 2002
J. Biol. Chem., Vol. 278, Issue 3, 1603-1611, January 17, 2003
The Yeast Mitochondrial Degradosome
ITS COMPOSITION, INTERPLAY BETWEEN RNA HELICASE AND RNase
ACTIVITIES AND THE ROLE IN MITOCHONDRIAL RNA METABOLISM*
Andrzej
Dziembowski §,
Jan
Piwowarski ,
Rafal
Hoser ,
Michal
Minczuk ,
Aleksandra
Dmochowska ,
Michel
Siep¶ ,
Hans
van der Spek¶,
Les
Grivell¶**, and
Piotr P.
Stepien 
From the Department of Genetics, Warsaw University
and Institute of Biochemistry and Biophysics, Polish Academy of
Sciences, Pawinskiego 5A, 02-106 Warsaw, Poland and the ¶ Section
for Molecular Biology, Swammerdam Institute for Life Sciences,
University of Amsterdam, Kruislaan 318, 1098 SM, Amsterdam, The Netherlands
The yeast mitochondrial degradosome (mtEXO) is an
NTP-dependent exoribonuclease involved in mitochondrial RNA
metabolism. Previous purifications suggested that it was composed of
three subunits. Our results suggest that the degradosome is composed of
only two large subunits: an RNase and a RNA helicase encoded by nuclear
genes DSS1 and SUV3, respectively, and that it
co-purifies with mitochondrial ribosomes. We have found that the
purified degradosome has RNA helicase activity that precedes and is
essential for exoribonuclease activity of this complex. The degradosome RNase activity is necessary for mitochondrial biogenesis but in vitro the degradosome without RNase activity is still able to unwind RNA. In yeast strains lacking degradosome components there is a
strong accumulation of mitochondrial mRNA and rRNA precursors not
processed at 3'- and 5'-ends. The observed accumulation of precursors
is probably the result of lack of degradation rather than direct
inhibition of processing. We suggest that the degradosome is a central
part of a mitochondrial RNA surveillance system responsible for
degradation of aberrant and unprocessed RNAs.
*
This work was supported in part by State Committee for
Scientific Research Grants 6P04 00319 and 6P04 01818, the Polish-French Center for Biotechnology of Plants EU Centre of Excellence in Molecular
Biology Grant ICA-CT-2000-70010, and Faculty of Biology Grant BW.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
§
Supported by a Foundation for Polish Science fellowship for young
scientists and an EMBO short-term fellowship for work at University of Amsterdam.
Current address: Dept. of Endocrinology and Reproduction,
Faculty of Medicine and Health Sciences, P. O. Box 1738, 3000 DR Rotterdam, The Netherlands. E-mail: siep@endov.fgg.eur.nl.
**
Current address: EMBO, Meyerhofstrasse 1, 69117 Heidelberg,
Germany. E-mail: grivell@embl-heidelberg.de.

To whom correspondence should be addressed: Dept. of Genetics,
Warsaw University and Institute of Biochemistry and Biophysics, Polish
Academy of Sciences, Pawinskiego 5A, 02-106 Warsaw, Poland. Tel.:
48-22-659-70-72 (ext. 22 40); Fax: 48-22-658-47-54; E-mail: stepien@ibb.waw.pl.
Copyright © 2003 by The American Society for Biochemistry and Molecular Biology, Inc.

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Copyright © 2003 by the American Society for Biochemistry and Molecular Biology.
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