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

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Loading and methylation of Sm proteins onto SMN Complexes

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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) Dreyfuss, G, Friesen, WJ	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 Dreyfuss, G, Charroux, B, Shevchenko, A, Mann, M, Pellizzoni, L, Perkinson, RA	J Cell Biol	1999
12065586	Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex Dreyfuss, G, Rappsilber, J, Baccon, J, Mann, M, Pellizzoni, L	J Biol Chem	2002
11748230	Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component Dreyfuss, G, Rappsilber, J, Baccon, J, Mann, M, Pellizzoni, L	J Biol Chem	2002
10725331	Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli. Dreyfuss, G, Charroux, B, Shevchenko, A, Mann, M, Pellizzoni, L, Perkinson, RA, Yong, J	J Cell Biol	2000
11714716	Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins Dreyfuss, G, Rappsilber, J, Mourelatos, Z, Abel, L, Mann, M, Gubitz, AK	J Biol Chem	2002