Gemin4 A Novel Component of the Smn Complex That Is Found in Both Gems and Nucleoli
作者： Bernard Charroux , Livio Pellizzoni , Robert A Perkinson , Jeongsik Yong , Andrej Shevchenko
关键词: Ribosome assembly 、 Cell biology 、 Spliceosomal snRNP assembly 、 SnRNP Biogenesis 、 SMN complex 、 SMN Complex Proteins 、 Survival of motor neuron 、 Biology 、 Molecular biology 、 snRNP 、 Preribosomal RNA
摘要: The survival of motor neurons (SMN) protein, the product neurodegenerative disease spinal muscular atrophy (SMA) gene, is localized both in cytoplasm and discrete nuclear bodies called gems. In compartments SMN part a large complex that contains several proteins including Gemin2 (formerly SIP1) DEAD box protein Gemin3. cytoplasm, associated with snRNP Sm core plays critical role spliceosomal assembly. nucleus, required for pre-mRNA splicing by serving regeneration spliceosomes. These functions are likely impaired cells SMA patients because they have reduced levels functional SMN. Here, we report identification nanoelectrospray mass spectrometry novel component name Gemin4. Gemin4 vivo through direct interaction tight Gemin3 suggests it could serve as cofactor this protein. also interacts directly proteins. Monoclonal antibodies against efficiently immunoprecipitate U snRNAs U1 U5 from Xenopus oocytes cytoplasm. Immunolocalization experiments show colocalized Interestingly, detected nucleoli, suggesting may function preribosomal RNA processing or ribosome
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