Recombinant
RabMAb

Anti-SMN/Gemin 1 antibody [EPR4430] (ab108424)

Overview

  • Product name
    Anti-SMN/Gemin 1 antibody [EPR4430]
    See all SMN/Gemin 1 primary antibodies
  • Description
    Rabbit monoclonal [EPR4430] to SMN/Gemin 1
  • Tested applications
    Suitable for: WB, IP, IHC-Pmore details
    Unsuitable for: Flow Cyt or ICC
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    corresponding to Human SMN/Gemin 1 aa 150-250.

  • Positive control
    • HeLa, HepG2, K562 cell lysates
  • General notes

    This product is a recombinant rabbit monoclonal antibody.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

Properties

Applications

Our Abpromise guarantee covers the use of ab108424 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 1/1000 - 1/10000. Predicted molecular weight: 32 kDa.
IP 1/10 - 1/100.
IHC-P 1/500.
  • Application notes
    Is unsuitable for Flow Cyt or ICC.
  • 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

    • All lanes : Anti-SMN/Gemin 1 antibody [EPR4430] (ab108424) at 1/1000 dilution

      Lane 1 : HeLa cell lysate
      Lane 2 : HepG2 cell lysate
      Lane 3 : K562 cell lysate

      Lysates/proteins at 10 µg per lane.


      Predicted band size : 32 kDa
    • ab108424 staining Gemin 1 in paraffin-embedded Human kidney tissue by Immunohistochemistry at dilution of 1:500.

    References

    ab108424 has not yet been referenced specifically in any publications.

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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