Survival of motor neuron protein
| Survival motor neuron protein | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| File:Protein SMN1 PDB 1g5v.png | |||||||||
| Identifiers | |||||||||
| Symbol | SMN | ||||||||
| Pfam | PF06003 | ||||||||
| Pfam clan | CL0049 | ||||||||
| InterPro | IPR010304 | ||||||||
| SCOP | 1mhn | ||||||||
| SUPERFAMILY | 1mhn | ||||||||
|
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The survival of motor neuron protein (SMN) is a complex of multiproteins involved in the assembly of snRNPs, the essential components of spliceosomal machinery.[2]It is encoded by the gene SMN1. A lack of SMN due to SMN1 deletion results in widespread splicing defects, especially in spinal motor neurons, and is one cause of spinal muscular atrophy.
SMN also functions in transcriptional regulation, telomerase regeneration and cellular trafficking.[3]
SMN is found in the cytoplasm of all animal cells and also in the nuclear gems. The protein complex includes at least six other proteins (gem-associated protein 2 - 7) [2]
Research also showed a possible role of SMN in neuronal migration and/or differentiation.[4]
Evolutionary conservation
SMN is evolutionary conserved including the Fungi kingdom, though only fungal organisms with a great number of introns has the smn gene (or the spf30 paralogue). Surprisingly, these are filamentous fungus which have mycelia, so suggesting analogy to the neuronal axons.[5]
See also
References
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- ↑ http://cat.inist.fr/?aModele=afficheN&cpsidt=17713178
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External links
- SMN protein (spinal muscular atrophy) at the US National Library of Medicine Medical Subject Headings (MeSH)
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