Results 1 to 10 of about 10,068 (192)

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
Janina Rafałowska, Dorota Sulejczak, Stanisław J Chrapusta, Roman Gadamski, Dorota Dziewulska   +4 more
doaj   +5 more sources

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

G3: Genes, Genomes, Genetics, 2019
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.
A. Gregory Matera, Amanda C. Raimer, Casey A. Schmidt, Jo A. Kelly, Gaith N. Droby, David Baillat, Sara ten Have, Angus I. Lamond, Eric J. Wagner, Kelsey M. Gray   +9 more
doaj   +5 more sources

Anti-survival of motor neuron antibodies in rheumatic and musculoskeletal diseases: prevalence, clinical associations, and biomarker potential, with novel insights into disease activity in SLE [PDF]

Inflammation and Regeneration
Background Anti-survival of motor neuron (SMN) antibodies have recently been identified in rheumatic and musculoskeletal diseases (RMDs), notably mixed connective tissue disease (MCTD).
Yuki Imai, Masaru Takeshita, Koji Suzuki, Hiroyuki Fukui, Kazunori Furuhashi, Kotaro Matsumoto, Jun Kikuchi, Keiko Yoshimoto, Yuko Kaneko   +8 more
doaj   +2 more sources

Tudor-based proteomic strategy pan-specifically enriches and identifies protein arginine methylation [PDF]

EMBO Reports
Protein arginine methylation is an important post-translational modification (PTM) in eukaryotes, regulating a variety of biological processes. Proteomic profiling of arginine methylation has advanced our understanding of its roles in biology and disease.
Lingzi Lu, Ting Li, Rou Zhang, Yutong Wang, Xiaoping Ye, Yixin Luo, Lingyu Sun, Liang Qi, Zilu Ye, Yang Mao, Yanqiu Yuan   +10 more
doaj   +2 more sources

Friend of Prmt1, FOP is a novel component of the nuclear SMN complex isolated using biotin affinity purification [PDF]

, 2014
SMN (survival motor neuron protein) complexes are essential for the biogenesis of uridine-rich small nuclear ribonucleoproteins (UsnRNPs). During the biogenesis, the SMN complexes bound to UsnRNPs are transported from the cytoplasm to the nucleus, and ...
Ishikawa, H. (Hiroki), Isobe, T. (Toshiaki), Izumikawa, K. (Keiichi), Miyazawa, N. (Naoki), Nakayama, H. (Hiroshi), Nobe, Y. (Yuko), Philipsen, J.N.J. (Sjaak), Takahashi, N. (Nozomi), Taoka, M. (Masato), Terukina, G. (Goro), Yamauchi, N. (Naoto), Yoshikawa, H. (Harunori)   +11 more
core   +10 more sources

Mechanism of assembly of snRNP cores assisted by ICln and the SMN complex in fission yeast

iScience, 2023
Summary: The spliceosomal snRNP cores, each comprised of a snRNA and a seven-membered Sm ring (D1/D2/F/E/G/D3/B), are assembled by twelve chaperoning proteins in human.
Yan Hu, Yan Hou, Shijie Zhou, Yingzhi Wang, Congcong Shen, Li Mu, Dan Su, Rundong Zhang   +7 more
doaj   +1 more source

Genetic Interactions between the Members of the SMN-Gemins Complex in Drosophila. [PDF]

PLoS ONE, 2015
The SMN-Gemins complex is composed of Gemins 2-8, Unrip and the survival motor neuron (SMN) protein. Limiting levels of SMN result in the neuromuscular disorder, spinal muscular atrophy (SMA), which is presently untreatable.
Rebecca M Borg, Rémy Bordonne, Neville Vassallo, Ruben J Cauchi   +3 more
doaj   +1 more source

WRAP53 is essential for Cajal body formation and for targeting the survival of motor neuron complex to Cajal bodies. [PDF]

PLoS Biology, 2010
The WRAP53 gene gives rise to a p53 antisense transcript that regulates p53. This gene also encodes a protein that directs small Cajal body-specific RNAs to Cajal bodies.
Salah Mahmoudi, Sofia Henriksson, Irene Weibrecht, Stephen Smith, Ola Söderberg, Staffan Strömblad, Klas G Wiman, Marianne Farnebo   +7 more
doaj   +1 more source

Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons. [PDF]

PLoS ONE, 2014
Spinal muscular atrophy (SMA) is caused by deficiency of the ubiquitously expressed survival motoneuron (SMN) protein. SMN is crucial component of a complex for the assembly of spliceosomal small nuclear ribonucleoprotein (snRNP) particles.
Benjamin Dombert, Rajeeve Sivadasan, Christian M Simon, Sibylle Jablonka, Michael Sendtner   +4 more
doaj   +1 more source

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   +1 more source