Recombinant
RabMAb

Recombinant Anti-SMN/Gemin 1 antibody [EPR4430] - BSA and Azide free (ab247668)

Overview

  • Product name

    Anti-SMN/Gemin 1 antibody [EPR4430] - BSA and Azide free
    See all SMN/Gemin 1 primary antibodies
  • Description

    Rabbit monoclonal [EPR4430] to SMN/Gemin 1 - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, IP, WBmore details
    Unsuitable for: Flow Cyt or ICC
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human SMN/Gemin 1 aa 150-250. The exact sequence is proprietary.

  • General notes

    Ab247668 is the carrier-free version of ab108424. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.

    Use our conjugation kits  for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

    ab247668 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

    Maxpar® is a trademark of Fluidigm Canada Inc.

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

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab247668 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
IHC-P Use at an assay dependent concentration. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
IP Use at an assay dependent concentration.
WB Use at an assay dependent concentration. Predicted molecular weight: 32 kDa.
  • 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

    References

    ab247668 has not yet been referenced specifically in any publications.

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