SMN complex
Definition
Target type: cellularcomponent
A protein complex that contains the survival motor neuron (SMN) protein and at least eight additional integral components, including the Gemin2-8 and Unrip proteins; the complex is found in the cytoplasm and in nuclear Gems, and is involved in spliceosomal snRNP assembly in the cytoplasm and in pre-mRNA splicing in the nucleus. [PMID:16434402, PMID:17023415]
The SMN complex is a multi-protein assembly critical for the assembly of snRNPs, the ribonucleoprotein complexes that are essential for pre-mRNA splicing. The complex is localized to the cytoplasm and the nucleus and is comprised of eight core proteins: SMN, Gemin2, Gemin3, Gemin4, Gemin5, Gemin6, Gemin7, and Gemin8. The SMN protein is the central component of the complex, and it acts as a scaffold for the assembly of other proteins. The Gemin proteins are involved in various aspects of snRNP assembly, including the binding of Sm proteins, the transport of snRNPs to the nucleus, and the regulation of snRNP biogenesis. The SMN complex is assembled in a stepwise manner, starting with the binding of Gemin2 and Gemin3 to SMN. This complex then recruits Gemin4 and Gemin5, followed by Gemin6 and Gemin7. Finally, Gemin8 is added to the complex, completing its assembly. Once the SMN complex is assembled, it can interact with snRNPs and facilitate their assembly. The complex also plays a role in the transport of snRNPs to the nucleus, where they are required for pre-mRNA splicing. Mutations in the SMN gene are responsible for spinal muscular atrophy, a debilitating neurodegenerative disease. The SMN complex is a highly dynamic structure, and its assembly and disassembly are tightly regulated. This regulation ensures that the complex is only assembled when needed and that it is disassembled when its function is no longer required. The SMN complex is a fascinating example of a multi-protein assembly that plays a vital role in cellular function. It is a complex structure that is precisely regulated and that is essential for the proper assembly and function of snRNPs. Further research is needed to fully understand the intricate mechanisms that govern the assembly and function of the SMN complex.'
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Proteins (2)
| Protein | Definition | Taxonomy |
|---|---|---|
| Survival motor neuron protein | A survival motor neuron protein that is encoded in the genome of human. [PRO:DAN, UniProtKB:Q16637] | Homo sapiens (human) |
| Gem-associated protein 4 | A gem-associated protein 4 that is encoded in the genome of human. [PRO:DNx, UniProtKB:P57678] | Homo sapiens (human) |
Compounds (3)
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| indoprofen | indoprofen : A monocarboxylic acid that is propionic acid in which one of the hydrogens at position 2 is substituted by a 4-(1-oxo-1,3-dihydroisoindol-2-yl)phenyl group. Initially used as an anti-inflammatory and analgesic, it was withdrawn from the market due to causing severe gastrointestinal bleeding. It has been subsequently found to increase production of the survival motor neuron protein. Indoprofen: A drug that has analgesic and anti-inflammatory properties. Following reports of adverse reactions including reports of carcinogenicity in animal studies it was withdrawn from the market worldwide. (From Martindale, The Extra Pharmacopoeia, 30th ed, p21) | gamma-lactam; isoindoles; monocarboxylic acid | EC 1.14.99.1 (prostaglandin-endoperoxide synthase) inhibitor; non-narcotic analgesic; non-steroidal anti-inflammatory drug |
| 1-[4-(4-bromophenyl)-2-thiazolyl]-4-piperidinecarboxamide | piperidinecarboxamide | ||
| dehydrocurvularin |