Overview

  • Product name

  • Description

    Rabbit polyclonal to SIX1
  • Host species

    Rabbit
  • Tested applications

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

    Reacts with: Mouse, Human
    Predicted to work with: Rat, Xenopus laevis, Gorilla
  • Immunogen

    Recombinant fragment corresponding to Human SIX1 aa 130-270.
    Sequence:

    CFKEKSRGVLREWYAHNPYPSPREKRELAEATGLTTTQVSNWFKNRRQRD RAAEAKERENTENNNSSSNKQNQLSPLEGGKPLMSSSEEEFSPPQSPDQN SVLLLQGNMGHARSSNYSLPGLTASQPSHGLQTHQHQLQDS


    Database link: Q15475

  • Positive control

    • Human skeletal muscle; U-251 cells; NIH-3T3 cell lysate; HEK293T overexpression lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab211359 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 1/250 - 1/500. Predicted molecular weight: 32 kDa.
IHC-P 1/500 - 1/1000. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Function

    May be involved in limb tendon and ligament development.
  • Tissue specificity

    Specifically expressed in skeletal muscle.
  • Involvement in disease

    Defects in SIX1 are the cause of deafness autosomal dominant type 23 (DFNA23) [MIM:605192]. A form of non-syndromic deafness characterized by prelingual, bilateral, symmetric hearing loss with a conductive component present in some but not all patients.
    Defects in SIX1 are the cause of branchiootic syndrome type 3 (BOS3) [MIM:608389]. BOS3 is a syndrome characterized by usually bilateral branchial cleft fistulas or cysts, sensorineural and/or conductive hearing loss, pre-auricular pits, and structural defects of the outer, middle or inner ear. Otic defects include malformed and hypoplastic pinnae, a narrowed external ear canal, bulbous internal auditory canal, stapes fixation, malformed and hypoplastic cochlea. Branchial and otic anomalies are as those seen in individuals with the branchiootorenal syndrome. However, renal anomalies are absent in branchiootic syndrome patients.
    Note=Defects in SIX1 could be a cause of branchiootorenal syndrome (BOR). BOR is an autosomal dominant disorder manifested by various combinations of preauricular pits, branchial fistulae or cysts, lacrimal duct stenosis, hearing loss, structural defects of the outer, middle, or inner ear, and renal dysplasia. Associated defects include asthenic habitus, long narrow facies, constricted palate, deep overbite, and myopia. Hearing loss may be due to mondini type cochlear defect and stapes fixation. Penetrance of BOR syndrome is high, although expressivity can be extremely variable.
  • Sequence similarities

    Belongs to the SIX/Sine oculis homeobox family.
    Contains 1 homeobox DNA-binding domain.
  • Cellular localization

    Nucleus.
  • Information by UniProt
  • Database links

  • Alternative names

    • BOS3 antibody
    • DFNA23 antibody
    • Homeobox protein SIX1 antibody
    • OTTHUMP00000179042 antibody
    • Sine oculis homeobox homolog 1 antibody
    • SIX homeobox 1 antibody
    • SIX1 antibody
    • SIX1_HUMAN antibody
    • TIP39 antibody
    see all

Images

  • All lanes : Anti-SIX1 antibody (ab211359) at 1/250 dilution

    Lane 1 : Negative control (vector only transfected HEK293T lysate)
    Lane 2 : SIX1 over-expression HEK293T lysate (Co-expressed with a C-terminal myc-DDK tag (~3.1 kDa) in mammalian HEK293T cells).

    Predicted band size: 32 kDa

  • Immunohistochemical analysis of paraffin-embedded Human skeletal muscle tissue labeling SIX1 with ab211359 at 1/500 dilution.

  • All lanes : Anti-SIX1 antibody (ab211359) at 1/250 dilution

    Lane 1 : NIH-3T3 cell lysate (Mouse embryonic fibroblast cells)
    Lane 2 : NBT-II cell lysate (Rat Wistar bladder tumour cells)

    Predicted band size: 32 kDa

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized U-251 MG cells labeling SIX1 with ab211359 at 4 µg/ml.

References

This product has been referenced in:

  • Wang P  et al. Dissecting the Global Dynamic Molecular Profiles of Human Fetal Kidney Development by Single-Cell RNA Sequencing. Cell Rep 24:3554-3567.e3 (2018). Read more (PubMed: 30257215) »
See 1 Publication for this product

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