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snRNAs contain specific SMN-binding domains that are essential for snRNP assembly.
Yong J
,
Golembe TJ
,
Battle DJ
,
Pellizzoni L
,
Dreyfuss G
.
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To serve in its function as an assembly machine for spliceosomal small nuclear ribonucleoprotein particles (snRNPs), the survival of motor neurons (SMN) protein complex binds directly to the Sm proteins and the U snRNAs. A specific domain unique to U1 snRNA, stem-loop 1 (SL1), is required for SMN complex binding and U1 snRNP Sm core assembly. Here, we show that each of the major spliceosomal U snRNAs (U2, U4, and U5), as well as the minor splicing pathway U11 snRNA, contains a domain to which the SMN complex binds directly and with remarkable affinity (low nanomolar concentration). The SMN-binding domains of the U snRNAs do not have any significant nucleotide sequence similarity yet they compete for binding to the SMN complex in a manner that suggests the presence of at least two binding sites. Furthermore, the SMN complex-binding domain and the Sm site are both necessary and sufficient for Sm core assembly and their relative positions are critical for snRNP assembly. These findings indicate that the SMN complex stringently scrutinizes RNAs for specific structural features that are not obvious from the sequence of the RNAs but are required for their identification as bona fide snRNAs. It is likely that this surveillance capacity of the SMN complex ensures assembly of Sm cores on the correct RNAs only and prevents illicit, potentially deleterious, assembly of Sm cores on random RNAs.
Baccon,
Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex.
2002, Pubmed
Baccon,
Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex.
2002,
Pubmed
Branlant,
U2 RNA shares a structural domain with U1, U4, and U5 RNAs.
1982,
Pubmed
Bühler,
Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophy.
1999,
Pubmed
,
Xenbase
Charroux,
Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems.
1999,
Pubmed
Charroux,
Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli.
2000,
Pubmed
,
Xenbase
Fischer,
Nucleo-cytoplasmic transport of U snRNPs: definition of a nuclear location signal in the Sm core domain that binds a transport receptor independently of the m3G cap.
1993,
Pubmed
,
Xenbase
Fischer,
An essential signaling role for the m3G cap in the transport of U1 snRNP to the nucleus.
1990,
Pubmed
,
Xenbase
Fischer,
The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis.
1997,
Pubmed
,
Xenbase
Friesen,
The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins.
2001,
Pubmed
Friesen,
Specific sequences of the Sm and Sm-like (Lsm) proteins mediate their interaction with the spinal muscular atrophy disease gene product (SMN).
2000,
Pubmed
Friesen,
A novel WD repeat protein component of the methylosome binds Sm proteins.
2002,
Pubmed
Friesen,
SMN, the product of the spinal muscular atrophy gene, binds preferentially to dimethylarginine-containing protein targets.
2001,
Pubmed
Gubitz,
Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins.
2002,
Pubmed
Hall,
Requirement of U12 snRNA for in vivo splicing of a minor class of eukaryotic nuclear pre-mRNA introns.
1996,
Pubmed
Hamm,
The trimethylguanosine cap structure of U1 snRNA is a component of a bipartite nuclear targeting signal.
1990,
Pubmed
,
Xenbase
Jarmolowski,
The determinants for Sm protein binding to Xenopus U1 and U5 snRNAs are complex and non-identical.
1993,
Pubmed
,
Xenbase
Jarmolowski,
Nuclear export of different classes of RNA is mediated by specific factors.
1994,
Pubmed
,
Xenbase
Jones,
Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarin.
2001,
Pubmed
,
Xenbase
Kambach,
Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs.
1999,
Pubmed
Lefebvre,
Identification and characterization of a spinal muscular atrophy-determining gene.
1995,
Pubmed
Liu,
Molecular characterization of the protein products of the fragile X syndrome gene and the survival of motor neurons gene.
1996,
Pubmed
Liu,
The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins.
1997,
Pubmed
Liu,
A novel nuclear structure containing the survival of motor neurons protein.
1996,
Pubmed
,
Xenbase
Lorson,
The domain encoded by exon 2 of the survival motor neuron protein mediates nucleic acid binding.
1998,
Pubmed
Lührmann,
Functions of U-snRNPs.
1990,
Pubmed
Lührmann,
Structure of spliceosomal snRNPs and their role in pre-mRNA splicing.
1990,
Pubmed
Mattaj,
Cap trimethylation of U snRNA is cytoplasmic and dependent on U snRNP protein binding.
1986,
Pubmed
,
Xenbase
Mattaj,
Nucleocytoplasmic transport and snRNP assembly.
1993,
Pubmed
Mattaj,
Ribonucleoprotein assembly: clues from spinal muscular atrophy.
1998,
Pubmed
,
Xenbase
Meister,
A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs.
2001,
Pubmed
,
Xenbase
Meister,
Methylation of Sm proteins by a complex containing PRMT5 and the putative U snRNP assembly factor pICln.
2001,
Pubmed
,
Xenbase
Meister,
Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs.
2002,
Pubmed
,
Xenbase
Meister,
Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins.
2000,
Pubmed
Melki,
Spinal muscular atrophy.
1997,
Pubmed
Montzka,
Additional low-abundance human small nuclear ribonucleoproteins: U11, U12, etc.
1988,
Pubmed
Mourelatos,
miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs.
2002,
Pubmed
Mourelatos,
SMN interacts with a novel family of hnRNP and spliceosomal proteins.
2001,
Pubmed
Pellizzoni,
The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1.
2001,
Pubmed
Pellizzoni,
Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component.
2002,
Pubmed
Pellizzoni,
A functional interaction between the survival motor neuron complex and RNA polymerase II.
2001,
Pubmed
Pellizzoni,
Essential role for the SMN complex in the specificity of snRNP assembly.
2002,
Pubmed
Pellizzoni,
SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.
1999,
Pubmed
Pellizzoni,
A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing.
1998,
Pubmed
Pillai,
Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing.
2003,
Pubmed
Plessel,
m3G cap hypermethylation of U1 small nuclear ribonucleoprotein (snRNP) in vitro: evidence that the U1 small nuclear RNA-(guanosine-N2)-methyltransferase is a non-snRNP cytoplasmic protein that requires a binding site on the Sm core domain.
1994,
Pubmed
,
Xenbase
Pu,
pICln inhibits snRNP biogenesis by binding core spliceosomal proteins.
1999,
Pubmed
,
Xenbase
Raker,
Spliceosomal U snRNP core assembly: Sm proteins assemble onto an Sm site RNA nonanucleotide in a specific and thermodynamically stable manner.
1999,
Pubmed
Stark,
Arrangement of RNA and proteins in the spliceosomal U1 small nuclear ribonucleoprotein particle.
2001,
Pubmed
Tarn,
Highly diverged U4 and U6 small nuclear RNAs required for splicing rare AT-AC introns.
1996,
Pubmed
Tarn,
A novel spliceosome containing U11, U12, and U5 snRNPs excises a minor class (AT-AC) intron in vitro.
1996,
Pubmed
Will,
Spliceosomal UsnRNP biogenesis, structure and function.
2001,
Pubmed
Yong,
Sequence-specific interaction of U1 snRNA with the SMN complex.
2002,
Pubmed
,
Xenbase
Zhang,
The Sm-like Hfq protein increases OxyS RNA interaction with target mRNAs.
2002,
Pubmed
