The SMN complex catalyzes the assembly of small nuclear ribonucleoproteins , the building blocks of the spliceosome, and thereby plays an important role in the splicing of cellular pre-mRNAs . Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP . In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP . To assemble core snRNPs, the SMN complex accepts the trapped 5Sm proteins from CLNS1A forming an intermediate . Within the SMN complex, SMN1 acts as a structural backbone and together with GEMIN2 it gathers the Sm complex subunits . Binding of snRNA inside 5Sm ultimately triggers eviction of the SMN complex, thereby allowing binding of SNRPD3 and SNRPB to complete assembly of the core snRNP . Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development . Also required for resolving RNA-DNA hybrids created by RNA polymerase II, that form R-loop in transcription terminal regions, an important step in proper transcription termination . May also play a role in the metabolism of small nucleolar ribonucleoprotein .

Survival motor neuron protein (Component of gems 1) (Gemin-1)

q16637

Human,Mouse,Rat

WB,IHC

WB 1:1000-1:5000
IHC 1:200-1:500

~32 kD

Rabbit

50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.

Store at -20°C. Stable for 12 months from date of receipt.