SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins. - Wikidata - awgldk - TriplyDB

SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.

SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.

http://www.wikidata.org/entity/Q35641208

about

Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assembly Gemin8 is required for the architecture and function of the survival motor neuron complex Gle1 functions during mRNA export in an oligomeric complex that is altered in human disease Toward an assembly line for U7 snRNPs: interactions of U7-specific Lsm proteins with PRMT5 and SMN complexes Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assembly SMN interacts with a novel family of hnRNP and spliceosomal proteins Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins.Symmetrical dimethylation of arginine residues in spliceosomal Sm protein B/B' and the Sm-like protein LSm4, and their interaction with the SMN protein.The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins Self-association of coilin reveals a common theme in nuclear body localization Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein The survival motor neuron protein forms soluble glycine zipper oligomers The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy Indoprofen upregulates the survival motor neuron protein through a cyclooxygenase-independent mechanism The RNA binding protein hnRNP Q modulates the utilization of exon 7 in the survival motor neuron 2 (SMN2) gene A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice A functional interaction between the survival motor neuron complex and RNA polymerase II SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta Structural basis for dimethylarginine recognition by the Tudor domains of human SMN and SPF30 proteins In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1 A novel WD repeat protein component of the methylosome binds Sm proteins Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway.Unrip is a component of SMN complexes active in snRNP assembly The SMN structure reveals its crucial role in snRNP assembly A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severity Regulation of SMN protein stability Residual Cajal bodies in coilin knockout mice fail to recruit Sm snRNPs and SMN, the spinal muscular atrophy gene product SMN in spinal muscular atrophy and snRNP biogenesis Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarin.Symmetrical dimethylarginine methylation is required for the localization of SMN in Cajal bodies and pre-mRNA splicing.Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs Inactivation of the SMN complex by oxidative stress Efficient SMN Rescue following Subcutaneous Tricyclo-DNA Antisense Oligonucleotide Treatment Lymphotropic Herpesvirus saimiri uses the SMN complex to assemble Sm cores on its small RNAs.

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P2860

SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.

http://www.wikidata.org/entity/Q35641208

description

1999 nî lūn-bûn

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1999年の論文

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1999年論文

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1999年論文

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1999年論文

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1999年論文

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1999年論文

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1999年论文

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1999年论文

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1999年论文

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name

SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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type

label

SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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prefLabel

SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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SMN mutants of spinal muscular ...... in binding to snRNP proteins.

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Proceedings of the National Academy of Sciences of the United States of America

P1476

SMN mutants of spinal muscular ...... e in binding to snRNP proteins

@en

P2093

B Charroux

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G Dreyfuss

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P2860

A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing

The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins

The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis

A novel nuclear structure containing the survival of motor neurons protein

A nuclear localization domain in the hnRNP A1 protein

Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q11.2-13.3

Identification and characterization of a spinal muscular atrophy-determining gene

Monoclonal antibodies to nucleic acid-containing cellular constituents: probes for molecular biology and autoimmune disease

Survival motor neuron gene transcript analysis in muscles from spinal muscular atrophy patients.

Missense mutation clustering in the survival motor neuron gene: a role for a conserved tyrosine and glycine rich region of the protein in RNA metabolism?

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11167-11172

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scholarly article

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20

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96

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Livio Pellizzoni

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12800520

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10500148

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P921

spinal muscular atrophy

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18005

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