Target
Human SMN1
Synonyms
SMN1 | BCD541 | Component of gems 1 | Gemin-1 | SMA2 | SMA4 | SMA@ | SMA1 | SMNC | SMNT | T-BCD541 | SMN | Survival motor neuron protein | Gemin 1 | GEMIN1 | SMA | SMA3
Reactivity
Human
(tested or 100% immunogen sequence identity)
Conjugations
Horseradish Peroxidase.
Also available
Unconjugated or conjugated with
FITC,
Biotin.
Immunogen
Recombinant Human Survival motor neuron protein protein (3-194AA)
Specificity
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).