ASA-B1736
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SMN1/2 Antibody
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Long Name | Antibody Type | Antibody Isotype | Host | Species Reactivity | Validated Applications | Purification |
survival of motor neuron 1, telomeric/survival of motor neuron 2, centromeric | Polyclonal | IgG | Rabbit | Human, Mouse, Rat | IHC-P, WB | Immunogen affinity purified. |
Immunogen | ||||||
A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2(22-52aa RRGTGQSDDSDIWDDTALIKAYDKAVASFKH), identical to the related mouse and rat sequences. |
Form | Lyophilized |
Size | 100 µg/vial |
Contents | Antibody is lyophilized with 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg NaN3. *carrier free antibody available upon request. |
Concentration | Reconstitute with 0.2 mL sterile dH2O (500 µg/ml final concentration). |
Storage | At -20 °C for 12 months, as supplied. Store reconstituted antibody at 2-8 °C for one month. For long-term storage, aliquot and store at -20 °C. Avoid repeated freezing and thawing. |
Gene | SMN1/2 |
Protein | Survival motor neuron protein |
Uniprot ID | Q16637 |
Function | 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. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs). |
Tissue Specificity | Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level). |
Sub-cellular localization | Cytoplasm. Nucleus, gem. Nucleus, Cajal body. Cytoplasmic granule. Cytoplasm, myofibril, sarcomere, Z line . Note: Colocalizes with Actn at the Z-line of skeletal muscle (By similarity). Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules. Colocalized and redistributed with ZPR1 from the cytoplasm to nuclear gems (Gemini of coiled bodies) and Cajal bodies. |
Sequence Similarities | Belongs to the SMN family. |
Aliases | Component of gems 1 antibody|Component of gems 2 antibody|Gemin 1 antibody|Gemin-1 antibody|SMA antibody|SMA1 antibody|SMA3 antibody|SMN antibody|SMN_HUMAN antibody|SMN1 antibody|SMN2 antibody|SMNC antibody| SMNT antibody|Survival motor neuron protein antibody|survival of motor neuron 1, telomeric antibody|survival of motor neuron 2, centromeric antibody |
Application | Concentration* | Species | Validated Using** |
Western blot | 0.1-0.5μg/ml | Human, Mouse, Rat | AssaySolutio's ECL kit |
Immunohistochemistry(Paraffin-embedded Section) | 0.5-1μg/ml | Human, Mouse, Rat | AssaySolutio's IHC/ICC Detection kit |