ArticleThe Journal of biological chemistry2009
Role of survival motor neuron complex components in small nuclear ribonucleoprotein assembly.
Article in The Journal of biological chemistry, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 25 citations in OpenAlex.
- EWAS in a polyphenol dense, DNA methylation-targeted, controlled diet and lifestyle study.Frontiers in nutrition · 2026Article
- A guide to the biogenesis and functions of endogenous small non-coding RNAs in animals.Nature reviews. Molecular cell biology · 2025Review
- Article
- The construction and validation of an RNA binding protein-related prognostic model for bladder cancer.BMC cancer · 2021Article
- Beyond a Binary Classification of Sex: An Examination of Brain Sex Differentiation, Psychopathology, and Genotype.Journal of the American Academy of Child and Adolescent Psychiatry · 2019Article
- UsnRNP biogenesis: mechanisms and regulation.Chromosoma · 2017Review
- SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.Human genetics · 2017Review
- Spinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction.Frontiers in molecular biosciences · 2017Review
- STRAP Acts as a Scaffolding Protein in Controlling the TLR2/4 Signaling Pathway.Scientific reports · 2016Article
- LARP6 Meets Collagen mRNA: Specific Regulation of Type I Collagen Expression.International journal of molecular sciences · 2016Review
- Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover.CNS neuroscience & therapeutics · 2014Review
- Identification of truncated forms of U1 snRNA reveals a novel RNA degradation pathway during snRNP biogenesis.Nucleic acids research · 2014Article
- Serine-threonine kinase receptor-associated protein (STRAP) regulates translation of type I collagen mRNAs.Molecular and cellular biology · 2013Article
- Human axonal survival of motor neuron (a-SMN) protein stimulates axon growth, cell motility, C-C motif ligand 2 (CCL2), and insulin-like growth factor-1 (IGF1) production.The Journal of biological chemistry · 2012Article
- Triptolide increases transcript and protein levels of survival motor neurons in human SMA fibroblasts and improves survival in SMA-like mice.British journal of pharmacology · 2012Article
- Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Nature reviews. Neuroscience · 2009Review
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Survival motor neuron (SMN) complex is essential for the biogenesis of the small nuclear ribonucleoprotein (snRNP) complex, although the complete role of each SMN complex component for the snRNP synthesis is largely unclear. We have identified an interaction between the two components Gemin2-Gemin7 using the mammalian two-hybrid system. In vitro stability assay revealed that the known SMN-Gemin7 interaction becomes stable in the presence of Gemin2 possibly via the identified Gemin2-Gemin7 interaction. Gemin7 knockdown revealed a decrease in snRNP assembly activity and a decrease in SmE protein, a component of snRNP, in the SMN complex, which was consistent with a previous discussion that the Gemin6-Gemin7 heterodimer may serve as a surrogate for the SmD3-SmB particle in forming a subcore, the intermediate complex for snRNP. Interestingly, we found that Unrip, but not Gemin8, can remove Gemin7 from the stable SMN-Gemin2-Gemin7 ternary complex. In an in vitro snRNP assembly assay using the Unrip knockdown and the untreated cell lysates, we revealed that there was a decrease in Gemin7 and increase in SmB/B' in the SMN complex observed in untreated cells during the assay, suggesting that the Gemin6-Gemin7 heterodimer in the subcore is exchanged by the SmD3-SmB particle to form snRNP. Surprisingly, these changes were not observed in the assay using the Unrip knockdown cell extracts, indicating the importance of Unrip in the formation of snRNP likely via removal of the Gemin6-Gemin7 from the SMN complex. Taken together, these results indicate that snRNP is synthesized by harmonization of the SMN complex components.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.