...Saki Ohazama; Akiko Fujimoto; Daisuke Konda; Ryota Yokoyama; Shinichi Nakagawa; Hiroshi Maita ABSTRACT Survival motor neuron protein (SMN), which is linked to spinal muscular atrophy, is a key component of the Gemin complex, which is essential for the assembly of small nuclear RNA-protein complexes...

...Luke W. Thompson; Kim D. Morrison; Sally L. Shirran; Ewout J. N. Groen; Thomas H. Gillingwater; Catherine H. Botting; Judith E. Sleeman ABSTRACT Spinal muscular atrophy (SMA) is an inherited neurodegenerative condition caused by a reduction in the amount of functional survival motor neuron (SMN...

... and actin networks can have catastrophic effects depending on cell size and polarity. The survival motor neuron (SMN) protein is ubiquitously involved in assembly of spliceosomal small nuclear ribonucleoprotein particles. Other SMN functions could, however, affect cellular activities driving asymmetrical...

...Eva Stejskalová; David Staněk ABSTRACT The nuclear SMN complex localizes to specific structures called nuclear gems. The loss of gems is a cellular marker for several neurodegenerative diseases. Here, we identify that the U1-snRNP-specific protein U1-70K localizes to nuclear gems, and we show...

..., including coilin, the survival of motor neuron protein (SMN) and the Sm protein D1 (SmD1, also known as SNRPD1). Moreover, depletion of Fam118B reduces splicing capacity and inhibits cell proliferation. In addition, Fam118B associates with coilin and SMN proteins. Fam118B depletion reduces symmetric...

... (SMN). Dysfunction of CB assembly occurs in spinal muscular atrophy (SMA). Here, we demonstrate that SMN is a SUMO1 target that has a small ubiquitin-related modifier (SUMO)-interacting motif (SIM)-like motif in the Tudor domain. The expression of SIM-like mutant constructs abolishes the interaction...

.... In particular, the SMN (survival motor neuron) protein complex is required for addition of the core Sm proteins to the snRNP. Insufficiency of SMN results in the inherited neurodegenerative condition, spinal muscular atrophy (SMA). Details of the physical organization of the cytoplasmic stages of snRNP...

[email protected] ) 24 2 2009 © The Company of Biologists Limited 2009 2009 Coilin SMN Small nuclear ribonucleoproteins Nuclear organization Cajal bodies (CBs) are subnuclear domains conserved in insects, including Drosophila ( Liu et al., 2006a ; Liu et al., 2006b ), yeast, plants...

...Benoît Renvoisé; Kevinee Khoobarry; Marie-Claude Gendron; Christian Cibert; Louis Viollet; Suzie Lefebvre Mutations of the survival motor neuron gene SMN1 cause the inherited disease spinal muscular atrophy (SMA). The ubiquitous SMN protein facilitates the biogenesis of spliceosomal small nuclear...

...Judith E. Sleeman; Laura Trinkle-Mulcahy; Alan R. Prescott; Stephen C. Ogg; Angus I. Lamond Analysis of stable cell lines expressing fluorescently tagged survival of motor neurons protein (SMN) and coilin shows striking differences in their dynamic behaviour, both in the nucleus and during mitosis...

... ). Functional architecture in the cell nucleus. Biochem. J. 356 , 297 -310. Fischer, U., Liu, Q. and Dreyfuss, G. ( 1997 ). The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis. Cell 90 , 1023 -1029. Frey, M. R. and Matera, A. G. ( 1995 ). Coiled bodies contain U7 small...

... that contain both p80-coilin and the survival of motor neurons protein (SMN) and not in bodies that contain p80-coilin but lack SMN. Treatment of cells with leptomycin B (LMB) inhibits both the accumulation of snRNPs in nuclear bodies and their subsequent accumulation in speckles. The formation of Cajal bodies...