Product nameAnti-SMN/Gemin 1 antibody
See all SMN/Gemin 1 primary antibodies
DescriptionRabbit polyclonal to SMN/Gemin 1
Tested applicationsSuitable for: WB, IHC-P, ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Macaque monkey
Recombinant fragment corresponding to Human SMN/Gemin 1 aa 3-194.
MSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFKH ALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAIW SEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANNI EQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFL
Database link: Q16637
- WB: A549 and HeLa whole cell lysate. IHC: Human testis tissue. ICC/IF: HepG2 cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.4
Preservative: 0.03% Proclin
Constituents: 50% Glycerol, PBS
Concentration information loading...
PurityProtein G purified
Purification notesPurity >95%
Our Abpromise guarantee covers the use of ab223068 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/5000.|
|IHC-P||1/20 - 1/200.|
|ICC/IF||1/50 - 1/200.|
FunctionThe SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. It may also play a role in the metabolism of snoRNPs.
Tissue specificityExpressed 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).
Involvement in diseaseDefects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 1 (SMA1) [MIM:253300]. Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Autosomal recessive forms are classified according to the age of onset, the maximum muscular activity achieved, and survivorship. The severity of the disease is mainly determined by the copy number of SMN2, a copy gene which predominantly produces exon 7-skipped transcripts and only low amount of full-length transcripts that encode for a protein identical to SMN1. Only about 4% of SMA patients bear one SMN1 copy with an intragenic mutation. SMA1 is a severe form, with onset before 6 months of age. SMA1 patients never achieve the ability to sit.
Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 2 (SMA2) [MIM:253550]. SMA2 is an autosomal recessive spinal muscular atrophy of intermediate severity, with onset between 6 and 18 months. Patients do not reach the motor milestone of standing, and survive into adulthood.
Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 3 (SMA3) [MIM:253400]. SMA3 is an autosomal recessive spinal muscular atrophy with onset after 18 months. SMA3 patients develop ability to stand and walk and survive into adulthood.
Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 4 (SMA4) [MIM:271150]. SMA4 is an autosomal recessive spinal muscular atrophy characterized by symmetric proximal muscle weakness with onset in adulthood and slow disease progression. SMA4 patients can stand and walk.
Sequence similaritiesBelongs to the SMN family.
Contains 1 Tudor domain.
Cellular localizationCytoplasm. Nucleus > gem. Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies.
- Information by UniProt
- BCD541 antibody
- Component of gems 1 antibody
- Gemin 1 antibody
All lanes : Anti-SMN/Gemin 1 antibody (ab223068) at 1/500 dilution
Lane 1 : A549 (human lung carcinoma cell line) whole cell lysate
Lane 2 : HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell lysate
All lanes : Goat polyclonal to rabbit IgG at 1/50000 dilution
Developed using the ECL technique.
Paraffin-embedded human testis tissue stained for SMN/Gemin 1 using ab223068 at 1/100 dilution in immunohistochemical analysis.
PFA-fixed, Triton X-100 permeabilized HepG2 (human liver hepatocellular carcinoma cell line) cells stained for SMN/Gemin 1(green) using ab223068 at 1/100 dilution in ICC/IF. Secondary: Alexa Fluor 488® conjugated Goat Anti-Rabbit IgG (H+L).
This product has been referenced in:
- Wu DM et al. MicroRNA-17 inhibition overcomes chemoresistance and suppresses epithelial-mesenchymal transition through a DEDD-dependent mechanism in gastric cancer. Int J Biochem Cell Biol 102:59-70 (2018). Read more (PubMed: 29953965) »