Reactome | Loading and methylation of Sm proteins onto SMN Complexes

Loading and methylation of Sm proteins onto SMN Complexes

Stable Identifier

R-HSA-191790

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

5/5

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Loading and methylation of Sm proteins onto SMN Complexes

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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
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
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
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