Results 1 to 10 of about 4,952 (108)

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

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

The U1 snRNP-specific protein U1C is a key regulator of SMN complex-mediated snRNP formation. [PDF]

J Biol Chem
Ngu DM, Myti S, Khan AA, Keita J, Moore T, Andega P, Aziz A, Raj R, Johnson-Winters K, Suh EH, So BR.   +10 more
europepmc   +3 more sources

A unique mechanism of snRNP core assembly [PDF]

Nature Communications
The assembly of most spliceosomal snRNP cores involves seven Sm proteins (D1/D2/F/E/G/D3/B) forming a ring around snRNA, typically requiring essential assembly chaperones like the SMN complex, associated with spinal muscular atrophy (SMA). Strikingly, in
Yingzhi Wang, Xiaoshuang Chen, Xi Kong, Yunfeng Chen, Zixi Xiang, Yue Xiang, Yan Hu, Yan Hou, Shijie Zhou, Congcong Shen, Li Mu, Dan Su, Rundong Zhang   +12 more
doaj   +2 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

The Gemin5 Protein of the SMN Complex Identifies snRNAs [PDF]

Molecular Cell, 2006
The survival of motor neurons protein (SMN) is part of a large complex that contains six other proteins, Gemins2-7. The SMN complex assembles the heptameric Sm protein core on small nuclear RNAs (snRNAs) and plays a critical role in the biogenesis of snRNPs, the major and essential components of mRNA splicing in eukaryotes.
Battle, Daniel J., Lau, Chi-Kong, Wan, Lili, Deng, Hongying, Lotti, Francesco, Dreyfuss, Gideon   +5 more
openaire   +2 more sources

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

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. SMN is part of a multi-protein complex that includes Gemin2, Gemin3, and Gemin4.
Gubitz, A.K., Mourelatos, Z., Abel, L., Rappsilber, J., Mann, M., Dreyfuss, G.   +5 more
openaire   +3 more sources

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