Results 1 to 10 of about 633,688 (189)

Functional mammalian spliceosomal complex E contains SMN complex proteins in addition to U1 and U2 snRNPs. [PDF]

Nucleic Acids Res, 2012
Copyright @ 2011 The Authors. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0), which permits unrestricted non-commercial use ...
Makarov EM, Owen N, Bottrill A, Makarova OV.   +3 more
europepmc   +7 more sources

Diverse expression of selected SMN complex proteins in humans with sporadic amyotrophic lateral sclerosis and in a transgenic rat model of familial form of the disease. [PDF]

PLoS One, 2014
BACKGROUND AND OBJECTIVE:There is circumstantial evidence linking sporadic amyotrophic lateral sclerosis (ALS) cases to a malfunction or deficit of a multimeric SMN complex that scrutinizes cellular RNAs; the core of this complex is survival motor neuron
Rafałowska J, Sulejczak D, Chrapusta SJ, Gadamski R, Dziewulska D.   +4 more
europepmc   +6 more sources

Composition of the Survival Motor Neuron (SMN) complex in Drosophila melanogaster [PDF]

G3: Genes, Genomes, Genetics, 2018
Spinal Muscular Atrophy (SMA) is caused by homozygous mutations in the human survival motor neuron 1 (SMN1) gene. SMN protein has a well-characterized role in the biogenesis of small nuclear ribonucleoproteins (snRNPs), core components of the spliceosome.
Baillat, David, Droby, Gaith N., Gray, Kelsey M., Kelly, Jo A., Lamond, Angus I., Matera, A. Gregory, Raimer, Amanda C., Schmidt, Casey A., Ten Have, Sara, Wagner, Eric J.   +9 more
core   +6 more sources

Proteomic analysis of the SMN complex reveals conserved and etiologic connections to the proteostasis network. [PDF]

Front RNA Res
IntroductionMolecular chaperones and co-chaperones are highly conserved cellular components that perform a variety of duties related to the proper three-dimensional folding of the proteome.
Matera AG, Steiner RE, Mills CA, McMichael BD, Herring LE, Garcia EL.   +5 more
europepmc   +3 more sources

The SMN complex drives structural changes in human snRNAs to enable snRNP assembly

Nature Communications, 2023
Spliceosomal snRNPs are multicomponent particles that undergo a complex maturation pathway. Human Sm-class snRNAs are generated as 3′-end extended precursors, which are exported to the cytoplasm and assembled together with Sm proteins into core RNPs by ...
Josef Pánek, Adriana Roithová, Nenad Radivojević, Michal Sýkora, Archana Bairavasundaram Prusty, Nicholas Huston, Han Wan, Anna Marie Pyle, Utz Fischer, David Staněk   +9 more
doaj   +2 more sources

SMN complex member Gemin3 self-interacts and has a functional relationship with ALS-linked proteins TDP-43, FUS and Sod1 [PDF]

Scientific Reports, 2019
The predominant motor neuron disease in infants and adults is spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS), respectively. SMA is caused by insufficient levels of the Survival Motor Neuron (SMN) protein, which operates as part of ...
Rebecca Cacciottolo, Joanna Ciantar, Maia Lanfranco, Rebecca Borg, Neville Vassallo, Rémy Bordonné, Ruben J. Cauchi   +6 more
openalex   +2 more sources

NUFIP and the HSP90/R2TP chaperone bind the SMN complex and facilitate assembly of U4-specific proteins [PDF]

Nucleic Acids Research, 2015
The Sm proteins are loaded on snRNAs by the SMN complex, but how snRNP-specific proteins are assembled remains poorly characterized. U4 snRNP and box C/D snoRNPs have structural similarities.
Jonathan Bizarro, Maxime Dodré, Alexandra Huttin, Bruno Charpentier, Florence Schlotter, Christiane Branlant, Céline Verheggen, Séverine Massenet, Édouard Bertrand   +8 more
openalex   +2 more sources

Gemin5, a Novel WD Repeat Protein Component of the SMN Complex That Binds Sm Proteins [PDF]

Journal of Biological Chemistry, 2002
The survival of motor neurons (SMN) protein is the product of the disease gene of spinal muscular atrophy and is found both in the cytoplasm and the nucleus, where it is concentrated in gems.
Amelie K. Gubitz, Zissimos P. Mourelatos, Linda Abel, Juri Rappsilber, Matthias Mann, Gideon Dreyfuss   +5 more
openalex   +2 more sources

Gemin4 is an essential gene in mice, and its overexpression in human cells causes relocalization of the SMN complex to the nucleoplasm

Biology Open, 2018
Gemin4 is a member of the Survival Motor Neuron (SMN) protein complex, which is responsible for the assembly and maturation of Sm-class small nuclear ribonucleoproteins (snRNPs).
Ingo D. Meier, Michael P. Walker, A. Gregory Matera   +2 more
doaj   +2 more sources

SECIS-binding protein 2 interacts with the SMN complex and the methylosome for selenoprotein mRNP assembly and translation [PDF]

Nucleic Acids Research, 2017
Selenoprotein synthesis requires the co-translational recoding of a UGASec codon. This process involves an RNA structural element, called Selenocysteine Insertion Sequence (SECIS) and the SECIS binding protein 2 (SBP2).
Anne-Sophie Gribling-Burrer, Michael Leichter, Laurence Wurth, Alexandra Huttin, Florence Schlotter, N. Troffer-Charlier, Vincent Cura, Martine Barkats, J. Cavarelli, Séverine Massenet, Christine Allmang   +10 more
openalex   +2 more sources