DATASHEET
Host:
Rabbit
Target Protein:
SMN1
Clonality:
Monoclonal
Isotype:
IgG
Entrez Gene:
6606
Swiss Prot:
Q16637
Source:
Recombinant protein within human SMN1 aa 1-200.
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C for 12 months.
Background:
The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. 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. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. 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 (snoRNPs).
VALIDATION IMAGES
Lane 1: SiHa Cells; Probed with SMN1 (6A1) Monoclonal Antibody (bsm-54395R) at 1:500, overnight at 4°C followed by a conjugated secondary antibody for 60 minutes at 37°C.
Paraformaldehyde-fixed and paraffin-embedded Rat Tesitis tissue incubated with SMN1 (6A1) Monoclonal Antibody (bsm-54395R) at 1:100, overnight at 4°C, followed by a conjugated secondary antibody and DAB staining. Counterstained with hematoxylin.
Paraformaldehyde-fixed and paraffin-embedded Mouse Brain tissue incubated with SMN1 (6A1) Monoclonal Antibody (bsm-54395R) at 1:100, overnight at 4°C, followed by a conjugated secondary antibody and DAB staining. Counterstained with hematoxylin.