Results 11 to 20 of about 10,068 (192)

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
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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
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The Spinal Muscular Atrophy Disease Gene Product, SMN, and Its Associated Protein SIP1 Are in a Complex with Spliceosomal snRNP Proteins [PDF]

Cell, 1997
Spinal muscular atrophy (SMA), one of the most common fatal autosomal recessive diseases, is characterized by degeneration of motor neurons and muscular atrophy. The SMA disease gene, termed Survival of Motor Neurons (SMN), is deleted or mutated in over 98% of SMA patients. The function of the SMN protein is unknown.
Liu, Qing, Fischer, Utz, Wang, Fan, Dreyfuss, Gideon   +3 more
openaire   +4 more sources

Drosophila SMN complex proteins Gemin2, Gemin3, and Gemin5 are components of U bodies

Experimental Cell Research, 2010
Uridine-rich small nuclear ribonucleoproteins (U snRNPs) play key roles in pre-mRNA processing in the nucleus. The assembly of most U snRNPs takes place in the cytoplasm and is facilitated by the survival motor neuron (SMN) complex. Discrete cytoplasmic RNA granules called U bodies have been proposed to be specific sites for snRNP assembly because they
Cauchi, R, Sanchez-Pulido, L, Liu, J
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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

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

Nucleic Acids Research, 2011
Spliceosomes remove introns from primary gene transcripts. They assemble de novo on each intron through a series of steps that involve the incorporation of five snRNP particles and multiple non-snRNP proteins. In mammals, all the intermediate complexes have been characterized on one transcript (MINX), with the exception of the very first, complex E. We
Makarov, E. M., Owen, N., Bottrill, A., Makarova, O. V.   +3 more
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Association of galectin-1 and galectin-3 with Gemin4 in complexes containing the SMN protein [PDF]

Nucleic Acids Research, 2001
In previous studies we showed that galectin-1 and galectin-3 are factors required for the splicing of pre-mRNA, as assayed in a cell-free system. Using a yeast two-hybrid screen with galectin-1 as bait, Gemin4 was identified as a putative interacting protein.
J W, Park, P G, Voss, S, Grabski, J L, Wang, R J, Patterson   +4 more
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A role for protein phosphatase PP1γ in SMN complex formation and subnuclear localization to Cajal bodies [PDF]

Journal of Cell Science, 2012
The spinal muscular atrophy (SMA) gene product SMN forms with Gemin2 to 8 and unrip the ubiquitous SMN complex that is required for the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs), their nuclear import and localization to subnuclear domain Cajal bodies (CBs). The concentration of the SMN complex and snRNPs in CBs are reduced upon
Renvoisé, B. (Benoît), Quérol, G. (Gwendoline), Verrier, E. (Eloi), Burlet, P. (Philippe), Lefebvre, S. (Suzie)   +4 more
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The distribution of SMN protein complex in human fetal tissues and its alteration in spinal muscular atrophy [PDF]

Human Molecular Genetics, 1998
Spinal muscular atrophy (SMA) is a common autosomal recessive neuromuscular disorder characterized by degeneration of motor neurons of the spinal cord and muscular atrophy. SMA is caused by alterations to the survival of motor neuron (SMN) gene, the function of which has hitherto been unclear.
P, Burlet, C, Huber, S, Bertrandy, M A, Ludosky, I, Zwaenepoel, O, Clermont, J, Roume, A L, Delezoide, J, Cartaud, A, Munnich, S, Lefebvre   +10 more
openaire   +2 more sources

SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta [PDF]

Human Molecular Genetics, 2002
The survival of motor neuron (SMN) protein is mutated in patients with spinal muscular atrophy (SMA). SMN is part of a multiprotein complex required for biogenesis of the Sm class of small nuclear ribonucleoproteins (snRNPs). Following assembly of the Sm core domain, snRNPs are transported to the nucleus via importin beta.
Usha, Narayanan, Jason K, Ospina, Mark R, Frey, Michael D, Hebert, A Gregory, Matera   +4 more
openaire   +2 more sources