Key features and details
- Rabbit polyclonal to SMN/Gemin 1
- Suitable for: WB, ICC/IF
- Reacts with: Human
- Isotype: IgG
Product nameAnti-SMN/Gemin 1 antibody
See all SMN/Gemin 1 primary antibodies
DescriptionRabbit polyclonal to SMN/Gemin 1
Tested applicationsSuitable for: WB, ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Cynomolgus monkey, Orangutan
Recombinant full length protein (His-tag) corresponding to Human SMN/Gemin 1 aa 2-294. Expressed in E.coli. N-terminal tag.
AMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFK HALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAI WSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANN IEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMP GPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICP DSLDDADALGSMLISWYMSGYHTGYYMGFRQNQKEGRCSHSLN
Database link: Q16637-1
- ICC/IF: HeLa cells. WB: HeLa, HEK-293T and HepG2 cell lysate; Recombinant human SMN/Gemin 1 protein.
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We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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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: 7.40
Preservative: 0.05% Proclin 300
Constituents: PBS, 50% Glycerol
Concentration information loading...
PurityProtein A purified
Purification notesAntigen-specific affinity chromatography followed by Protein A affinity chromatography.
Our Abpromise guarantee covers the use of ab231351 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.5 - 2 µg/ml.|
|ICC/IF||Use a concentration of 5 - 20 µg/ml.|
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
FITC staining of formalin-fixed HeLa (human epithelial cell line from cervix adenocarcinoma) cells labeling SMN/Gemin 1 (green)using ab231351 at 20 μg/ml in ICC/IF.
All lanes : Anti-SMN/Gemin 1 antibody (ab231351) at 3 µg/ml
Lane 1 : HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell lysate
Lane 2 : HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) whole cell lysate
Lane 3 : HepG2 (human liver hepatocellular carcinoma cell line) whole cell lysate
All lanes : HRP-Linked Guinea pig Anti-Rabbit Ab at 1/2000 dilution
Anti-SMN/Gemin 1 antibody (ab231351) at 3 µg/ml + Recombinant human SMN/Gemin 1 protein
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab231351 has not yet been referenced specifically in any publications.