Loading and methylation of Sm proteins onto SMN Complexes

Stable Identifier
R-HSA-191790
Type
Reaction [transition]
Species
Homo sapiens
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The survival of motor neurons (SMN) complex binds to Sm proteins and small nuclear RNAs (snRNAs) in the cytoplasm. Sm is part the SMN multiprotein complex that contains Gemins 2 – 7, including the DEAD-box RNA helicase Gemin3. The binding of the SMN complex to the snRNAs depends on the presence of specific, high-affinity (nanomolar) binding domains in the snRNAs. The SMN complex binds the Sm proteins through the Sm domains interaction with the Gemins, the TUDOR domain, and through unique arginine- and glycine-rich (RG) domains found in three of these, SmB, SmD1 and SmD3. The association with RG domains is strongly enhanced by the post-translational symmetric dimethylation of specific arginines in these domains, a process that is carried out by the methylosome (JBP1 or PRMT5) complex.

Literature References
PubMed ID Title Journal Year
10601333 Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems

Charroux, B, Pellizzoni, L, Perkinson, RA, Shevchenko, A, Mann, M, Dreyfuss, G

J Cell Biol 1999
10851237 Specific sequences of the Sm and Sm-like (Lsm) proteins mediate their interaction with the spinal muscular atrophy disease gene product (SMN)

Friesen, WJ, Dreyfuss, G

J Biol Chem 2000
12065586 Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex

Baccon, J, Pellizzoni, L, Rappsilber, J, Mann, M, Dreyfuss, G

J Biol Chem 2002
11748230 Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component

Pellizzoni, L, Baccon, J, Rappsilber, J, Mann, M, Dreyfuss, G

J Biol Chem 2002
10725331 Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli.

Charroux, B, Pellizzoni, L, Perkinson, RA, Yong, J, Shevchenko, A, Mann, M, Dreyfuss, G

J Cell Biol 2000
11714716 Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins

Gubitz, AK, Mourelatos, Z, Abel, L, Rappsilber, J, Mann, M, Dreyfuss, G

J Biol Chem 2002
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Title
protein-arginine N-methyltransferase activity of Methylosome [cytosol]
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