| Name | snRNPs |
|---|---|
| Synonyms | G7b; LSM 2; LSM2; Protein G7b; Small nuclear ribonuclear protein D homolog; SnRNP core SM like protein SM x5; U6 snRNA associated Sm like protein LSm2; YBL026W… |
| Name | SMA |
|---|---|
| CAS | sodium 2-chloroacetate |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 18485864 | Talbot K, Davies KE: Is good housekeeping the key to motor neuron survival? . Cell. 2008 May 16;133(4):572-4. Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. |
82(1,1,1,2) | Details |
| 19062530 | Baranov VS, Kiselev AV, Vakharlovskii VG, Zhelezniakova GIu, Komantsev VN, Malysheva OV, Glotov AS, Ivashchenko TE, Baranov AN: [Molecular genetic basis of proximal spinal muscular atrophy and experience in its pharmaceutical treatment]. Genetika. 2008 Oct;44(10):1325-37. The structures of the SMN1 gene and SMN2 pseudogene, mutations distorting the SMN1 function, the structure and functions of the Smn neurotrophic protein, its role in biogenesis of small nuclear ribonucleoproteins (snRNPs), and the principles and prdblems of molecular diagnosis in SMA are described. |
31(0,1,1,1) | Details |
| 17895963 | Gabanella F, Butchbach ME, Saieva L, Carissimi C, Burghes AH, Pellizzoni L: Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS One. 2007 Sep 26;2(9):e921. |
8(0,0,0,8) | Details |
| 19329542 | Workman E, Saieva L, Carrel TL, Crawford TO, Liu D, Lutz C, Beattie CE, Pellizzoni L, Burghes AH: A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice. Hum Mol Genet. 2009 Jun 15;18(12):2215-29. Epub 2009 Mar 27. |
6(0,0,0,6) | Details |
| 18000835 | McWhorter ML, Boon KL, Horan ES, Burghes AH, Beattie CE: The SMN binding protein Gemin2 is not involved in motor axon outgrowth. Dev Neurobiol. 2008 Feb 1;68(2):182-94. It has been hypothesized that SMN may have a dual function: a role in snRNP assembly and a novel function that affects axons. |
3(0,0,0,3) | Details |
| 19735367 | Clelland AK, Kinnear NP, Oram L, Burza J, Sleeman JE: The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells. Traffic. 2009 Nov;10(11):1585-98. Epub 2009 Aug 4. CBs are the first site of accumulation of newly assembled splicing snRNPs (small nuclear ribonucleoproteins) following their import into the nucleus, before they form their steady-state localization in nuclear splicing speckles. |
3(0,0,0,3) | Details |
| 19129172 | Renvoise B, Colasse S, Burlet P, Viollet L, Meier UT, Lefebvre S: The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy. Hum Mol Genet. 2009 Apr 1;18(7):1181-9. Epub 2009 Jan 7. The assembly machinery containing SMN is implicated in the biogenesis of the spliceosomal small nuclear ribonucleoproteins (snRNPs). |
2(0,0,0,2) | Details |
| 19584893 | Burghes AH, Beattie CE: Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?. Nat Rev Neurosci. 2009 Aug;10(8):597-609. Epub 2009 Jul 8. SMN, together with partner proteins, functions in the assembly of small nuclear ribonucleoproteins (snRNPs), which are important for pre-mRNA splicing. |
2(0,0,0,2) | Details |
| 19928837 | Fuller HR, Man NT, Lam le T, Thanh le T, Keough RA, Asperger A, Gonda TJ, Morris GE: The SMN interactome includes Myb-binding protein 1a. . J Proteome Res. 2010 Jan;9(1):556-63. One-hundred and one proteins were detected with a p value of <0.05, SMN, gemins and U snRNPs being the dominant "hits". |
1(0,0,0,1) | Details |
| 20053895 | Coady TH, Lorson CL: Trans-splicing-mediated improvement in a severe mouse model of spinal muscular atrophy. J Neurosci. 2010 Jan 6;30(1):126-30. Here, we demonstrate that in vivo delivery of the optimized trans-splicing vector increases an important SMN-dependent activity, snRNP assembly, in disease-relevant tissue in the SMA mouse model. |
1(0,0,0,1) | Details |
| 19343312 | Rossoll W, Bassell GJ: Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes. Results Probl Cell Differ. 2009;48:289-326. Spinal muscular atrophy (SMA) is a neurodegenerative disease that results from loss of function of the SMN1 gene, encoding the ubiquitously expressed survival of motor neuron (SMN) protein, a protein best known for its housekeeping role in the SMN-Gemin multiprotein complex involved in spliceosomal small nuclear ribonucleoprotein (snRNP) assembly. |
1(0,0,0,1) | Details |
| 18941511 | Coady TH, Baughan TD, Shababi M, Passini MA, Lorson CL: Development of a single vector system that enhances trans-splicing of SMN2 transcripts. PLoS One. 2008;3(10):e3468. Epub 2008 Oct 22. The ASO-tsRNA vector significantly elevated SMN levels in primary SMA patient fibroblasts, within the central nervous system of SMA mice and increased SMN-dependent in vitro snRNP assembly. |
1(0,0,0,1) | Details |
| 19286363 | Chari A, Paknia E, Fischer U: The role of RNP biogenesis in spinal muscular atrophy. . Curr Opin Cell Biol. 2009 Jun;21(3):387-93. Epub 2009 Mar 13. This ubiquitous factor is part of a complex that mediates the formation of spliceosomal snRNPs. |
1(0,0,0,1) | Details |
| 18675250 | Lorson MA, Dickson AM, Shaw DJ, Todd AG, Young EC, Morse R, Wolstencroft C, Lorson CL, Young PJ: Identification and characterisation of a nuclear localisation signal in the SMN associated protein, Gemin4. Biochem Biophys Res Commun. 2008 Oct 10;375(1):33-7. Epub 2008 Jul 31. Gemin4 is a ubiquitously expressed multifunctional protein that is involved in U snRNP assembly, apoptosis, nuclear/cytoplasmic transportation, transcription, and RNAi pathways. |
1(0,0,0,1) | Details |
| 19464282 | Lee L, Davies SE, Liu JL: The spinal muscular atrophy protein SMN affects Drosophila germline nuclear organization through the U body-P body pathway. Dev Biol. 2009 Aug 1;332(1):142-55. Epub 2009 May 21. SMN is critical for small nuclear ribonucleoprotein (snRNP) assembly. |
1(0,0,0,1) | Details |
| 18485868 | Zhang Z, Lotti F, Dittmar K, Younis I, Wan L, Kasim M, Dreyfuss G: SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell. 2008 May 16;133(4):585-600. The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. |
1(0,0,0,1) | Details |
| 18629520 | Simic G: Pathogenesis of proximal autosomal recessive spinal muscular atrophy. . Acta Neuropathol. 2008 Sep;116(3):223-34. Epub 2008 Jul 16. In this review, new findings regarding the dual cellular role of the SMN protein (translocation of beta-actin to axonal growth cones and snRNP biogenesis/pre-mRNA splicing) were integrated with recent data obtained by detailed neuropathological examination of SMA and control subjects. |
1(0,0,0,1) | Details |