Overview

  • Product name

  • Description

    Rabbit polyclonal to SMN/Gemin 1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Cynomolgus monkey, Orangutan
  • Immunogen

    Recombinant full length protein (His-tag) corresponding to Human SMN/Gemin 1 aa 2-294. Expressed in E.coli. N-terminal tag.
    Sequence:

    AMSSGGSGGGVPEQEDSVLFRRGTGQSDDSDIWDDTALIKAYDKAVASFK HALKNGDICETSGKPKTTPKRKPAKKNKSQKKNTAASLQQWKVGDKCSAI WSEDGCIYPATIASIDFKRETCVVVYTGYGNREEQNLSDLLSPICEVANN IEQNAQENENESQVSTDESENSRSPGNKSDNIKPKSAPWNSFLPPPPPMP GPRLGPGKPGLKFNGPPPPPPPPPPHLLSCWLPPFPSGPPIIPPPPPICP DSLDDADALGSMLISWYMSGYHTGYYMGFRQNQKEGRCSHSLN


    Database link: Q16637-1

  • Positive control

    • ICC/IF: HeLa cells. WB: HeLa, HEK-293T and HepG2 cell lysate; Recombinant human SMN/Gemin 1 protein.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped 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 buffer

    pH: 7.4
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Protein A purified
  • Purification notes

    Antigen-specific affinity chromatography followed by Protein A affinity chromatography.
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

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.

Application Abreviews Notes
WB Use a concentration of 0.5 - 2 µg/ml.
ICC/IF Use a concentration of 5 - 20 µg/ml.

Target

  • Function

    The 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 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).
  • Involvement in disease

    Defects 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 similarities

    Belongs to the SMN family.
    Contains 1 Tudor domain.
  • Cellular localization

    Cytoplasm. Nucleus > gem. Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies.
  • Information by UniProt
  • Database links

  • Alternative names

    • BCD541 antibody
    • Component of gems 1 antibody
    • Gemin 1 antibody
    • Gemin-1 antibody
    • OTTHUMP00000125198 antibody
    • OTTHUMP00000223567 antibody
    • OTTHUMP00000223568 antibody
    • OTTHUMP00000224066 antibody
    • OTTHUMP00000226924 antibody
    • SMA 1 antibody
    • SMA 2 antibody
    • SMA 3 antibody
    • SMA 4 antibody
    • SMA antibody
    • SMA@ antibody
    • SMA1 antibody
    • SMA2 antibody
    • SMA3 antibody
    • SMA4 antibody
    • SMN antibody
    • SMN_HUMAN antibody
    • SMN1 antibody
    • SMN2 antibody
    • SMNT antibody
    • Survival motor neuron protein antibody
    • Survival of motor neuron 1, telomeric antibody
    • T-BCD541 antibody
    see all

Images

  • 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

    Secondary
    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

References

ab231351 has not yet been referenced specifically in any publications.

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