The SMN structure reveals its crucial role in snRNP assembly

scientific article

The SMN structure reveals its crucial role in snRNP assembly is …
instance of (P31):
scholarly articleQ13442814
retracted paperQ45182324

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P356DOI10.1093/HMG/DDU734
P698PubMed publication ID25561692

P50authorCraig MageeQ47503982
P2093author name stringJames P Allen
Christian L Lorson
Philip J Young
Chenda O Seng
P2860cites workThe Gemin5 protein of the SMN complex identifies snRNAsQ24297645
Structure of the spliceosomal U4 snRNP core domain and its implication for snRNP biogenesisQ24300255
Functional organization of the Sm core in the crystal structure of human U1 snRNPQ24309502
An assembly chaperone collaborates with the SMN complex to generate spliceosomal SnRNPsQ24311843
The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteinsQ24316085
Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assemblyQ24319038
The survival motor neuron protein forms soluble glycine zipper oligomersQ24600623
PHENIX: a comprehensive Python-based system for macromolecular structure solutionQ24654617
PROCHECK: a program to check the stereochemical quality of protein structuresQ26778411
SMN tudor domain structure and its interaction with the Sm proteinsQ27629094
High-resolution X-ray and NMR structures of the SMN Tudor domain: conformational variation in the binding site for symmetrically dimethylated arginine residuesQ27640685
Crystal structure of human spliceosomal U1 snRNP at 5.5 Å resolutionQ27654469
The neurobiology of childhood spinal muscular atrophy.Q41489423
Mutation update of spinal muscular atrophy in Spain: molecular characterization of 745 unrelated patients and identification of four novel mutations in the SMN1 gene.Q44669542
Analysis of point mutations in the SMN1 gene in SMA patients bearing a single SMN1 copy.Q45867775
An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMNQ73295491
A role for polyproline motifs in the spinal muscular atrophy protein SMN. Profilins bind to and colocalize with smn in nuclear gemsQ73297574
SMN oligomerization defect correlates with spinal muscular atrophy severityQ74557706
Gemin2 plays an important role in stabilizing the survival of motor neuron complexQ79794565
SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMNQ81385781
Structural basis of assembly chaperone- mediated snRNP formationQ27683788
Coot: model-building tools for molecular graphicsQ27860505
SFCHECK: a unified set of procedures for evaluating the quality of macromolecular structure-factor data and their agreement with the atomic modelQ27861069
The CCP4 suite: programs for protein crystallographyQ27861090
Gemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesisQ28115894
The spliceosome: design principles of a dynamic RNP machineQ28131809
Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophyQ28137718
The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 bindingQ28138530
The C-terminal RG dipeptide repeats of the spliceosomal Sm proteins D1 and D3 contain symmetrical dimethylarginines, which form a major B-cell epitope for anti-Sm autoantibodiesQ28140527
SMN, the product of the spinal muscular atrophy gene, binds preferentially to dimethylarginine-containing protein targetsQ28199238
LSm proteins form heptameric rings that bind to RNA via repeating motifsQ28264182
Regulation of SMN protein stabilityQ28304878
Identification and characterization of a spinal muscular atrophy-determining geneQ29547495
A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophyQ29617367
Practically useful: what the Rosetta protein modeling suite can do for youQ30386768
Inactivation of the SMN complex by oxidative stressQ33355418
Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Q33785933
Specific sequences of the Sm and Sm-like (Lsm) proteins mediate their interaction with the spinal muscular atrophy disease gene product (SMN).Q33905442
Specific sequence features, recognized by the SMN complex, identify snRNAs and determine their fate as snRNPsQ34230914
Essential role for the SMN complex in the specificity of snRNP assemblyQ34529450
Splicing in disease: disruption of the splicing code and the decoding machineryQ34582131
Identification of proximal spinal muscular atrophy carriers and patients by analysis of SMNT and SMNC gene copy numberQ35250646
SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.Q35641208
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.Q36602121
Chaperoning ribonucleoprotein biogenesis in health and diseaseQ36777301
Evolution of an RNP assembly system: a minimal SMN complex facilitates formation of UsnRNPs in Drosophila melanogasterQ36786870
snRNAs contain specific SMN-binding domains that are essential for snRNP assemblyQ37012661
Evolutionary diversification of the Sm family of RNA-associated proteinsQ37238158
SMN and Gemins: 'we are family' … or are we?: insights into the partnership between Gemins and the spinal muscular atrophy disease protein SMN.Q37800873
Deciphering arginine methylation: Tudor tells the tale.Q37931616
Tudor: a versatile family of histone methylation 'readers'.Q38137526
The intriguing case of motor neuron disease: ALS and SMA come closer.Q38164393
ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN.Q39150934
Mislocalised FUS mutants stall spliceosomal snRNPs in the cytoplasmQ39176818
Molecular and functional analysis of intragenic SMN1 mutations in patients with spinal muscular atrophy.Q40484524
Tudor domains in proteins that interact with RNA.Q41361779
P433issue8
P407language of work or nameEnglishQ1860
P304page(s)2138-46
P577publication date2015-04-15
P1433published inHuman Molecular GeneticsQ2720965
P1476titleThe SMN structure reveals its crucial role in snRNP assembly
P478volume24

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cites work (P2860)
Q42289539A critical examination of the recently reported crystal structures of the human SMN protein.
Q93178436Disruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions
Q36389484How do SMA-linked mutations of SMN1 lead to structural/functional deficiency of the SMA protein?
Q51636016Mutation Spectrum of the Survival of Motor Neuron 1 and Functional Analysis of Variants in Chinese Spinal Muscular Atrophy.
Q48646595Retraction notice: the SMN structure reveals its crucial role in snRNP assembly
Q47885235SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.
Q38684609Seeing but not believing: the structure of glycerol dehydrogenase initially assumed to be the structure of a survival protein from Salmonella typhimurium
Q36613857Spatial regulation of cytoplasmic snRNP assembly at the cellular level
Q30398280Structure determination of contaminant proteins using the MarathonMR procedure
Q30008890Targeting the SH3 domain of human osteoclast-stimulating factor with rationally designed peptoid inhibitors
Q46255967The science is in the data.

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