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Rabbit Anti-Gemin 8 antibody
Gemin8 is a 242 amino acid protein encoded by the mouse gene Gemin8. Gemin8, along with Gemins 2-7 and unrip, is a major component of the large multiprotein survival of motor neurons (SMN) complex. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease spinal muscular atrophy, is also part of the SMN complex. The SMN complex is a modular composition of proteins with SMN, Gemin8, and Gemin7 in its center. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. Gemin8 binds directly to SMN and mediates its interaction with the Gemin6/Gemin7 heterodimer. Importantly, the loss of Gemin6, Gemin7, and Unrip interaction with SMN as a result of Gemin8 knockdown affects snRNP assembly by impairing the SMN complex association with Sm proteins but not with snRNAs. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Function:
Gemin 8 forms a large macromolecular complex which includes other gemin proteins, SMN (survival motor neuron) protein and Unrip. This complex is required for assembly of small nuclear ribonucleoproteins (snRNPs) from Sm proteins and snRNAs. Gemin 8 mediates the binding of SMN with gemin 6 and gemin 7.
Subcellular Location:
Cytoplasmic and Nuclear
SWISS:
Q9NWZ8
Gene ID:
54960
Database links:
Entrez Gene: 54960 Human
SwissProt: Q9NWZ8 Human
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