製品の概要

  • 製品名Anti-PAX3 antibody [C2]
    PAX3 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal [C2] to PAX3
  • アプリケーション適用あり: IHC-P, Flow Cyt, WBmore details
  • 種交差性
    交差種: Human, Quail
  • 免疫原

    Synthetic peptide (quail) from the C-terminus

  • ポジティブ・コントロール
    • 624 Mel nuclear lysate; Human skeletal muscle and Human skin tissue

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab69856 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
IHC-P Use a concentration of 10 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Flow Cyt Use 0.5-1µg for 106 cells. ab170191-Mouse monoclonal IgG2a, is suitable for use as an isotype control with this antibody.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 53 kDa (predicted molecular weight: 53 kDa).

ターゲット情報

  • 機能Probable transcription factor associated with development of alveolar rhabdomyosarcoma.
  • 関連疾患Defects in PAX3 are the cause of Waardenburg syndrome type 1 (WS1) [MIM:193500]. WS1 is an autosomal dominant disorder characterized by wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum), pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma and sensorineural deafness. The syndrome shows variable clinical expression and some affected individuals do not manifest hearing impairment.
    Defects in PAX3 are the cause of Waardenburg syndrome type 3 (WS3) [MIM:148820]; also known as Klein-Waardenburg syndrome or Waardenburg syndrome with upper limb anomalies or white forelock with malformations. WS3 is a very rare autosomal dominant disorder, which shares many of the characteristics of WS1. Patients additionally present with musculoskeletal abnormalities.
    Defects in PAX3 are the cause of craniofacial-deafness-hand syndrome (CDHS) [MIM:122880]. CDHS is thought to be an autosomal dominant disease which comprises absence or hypoplasia of the nasal bones, hypoplastic maxilla, small and short nose with thin nares, limited movement of the wrist, short palpebral fissures, ulnar deviation of the fingers, hypertelorism and profound sensory-neural deafness.
    Defects in PAX3 are a cause of rhabdomyosarcoma type 2 (RMS2) [MIM:268220]. It is a form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchimal cells and exhibiting differentiation along rhabdomyoblastic lines. Rhabdomyosarcoma is one of the most frequently occurring soft tissue sarcomas and the most common in children. It occurs in four forms: alveolar, pleomorphic, embryonal and botryoidal rhabdomyosarcomas. Note=A chromosomal aberration involving PAX3 is found in rhabdomyosarcoma. Translocation (2;13)(q35;q14) with FOXO1. The resulting protein is a transcriptional activator.
    Note=A chromosomal aberration involving PAX3 is a cause of rhabdomyosarcoma. Translocation t(2;2)(q35;p23) with NCOA1 generates the NCOA1-PAX3 oncogene consisting of the N-terminus part of PAX3 and the C-terminus part of NCOA1. The fusion protein acts as a transcriptional activator. Rhabdomyosarcoma is the most common soft tissue carcinoma in childhood, representing 5-8% of all malignancies in children.
  • 配列類似性Belongs to the paired homeobox family.
    Contains 1 homeobox DNA-binding domain.
    Contains 1 paired domain.
  • 細胞内局在Nucleus.
  • Information by UniProt
  • 参照データベース
  • 別名
    • CDHS antibody
    • HUP 2 antibody
    • HUP2 antibody
    • MGC120381 antibody
    • MGC120382 antibody
    • MGC120383 antibody
    • MGC120384 antibody
    • MGC134778 antibody
    • Paired box 3 antibody
    • Paired box gene 3 antibody
    • Paired box homeotic gene 3 antibody
    • Paired box protein Pax 3 antibody
    • Paired box protein Pax-3 antibody
    • Paired box protein Pax3 antibody
    • Paired domain gene 3 antibody
    • Paired domain gene HuP2 antibody
    • PAX 3 antibody
    • Pax3 antibody
    • PAX3/FKHR fusion gene antibody
    • PAX3_HUMAN antibody
    • Sp antibody
    • splotch antibody
    • Waardenburg syndrome 1 antibody
    • WS 1 antibody
    • WS1 antibody
    • WS3 antibody
    see all

Anti-PAX3 antibody [C2] 画像

  • All lanes : Anti-PAX3 antibody [C2] (ab69856) at 1 µg/ml

    Lane 1 : Marker
    Lane 2 : 624 Mel nuclear lysate

    Developed using the ECL technique

    Predicted band size : 53 kDa
    Observed band size : 53 kDa
  • ab109691, at 10 µg/ml, staining PAX3 in formalin-fixed, paraffin-embedded Human Skeletal muscle tissue by Immunohistochemistry.
  • ab109691, at 10 µg/ml, staining PAX3 in formalin-fixed, paraffin-embedded Human Skin tissue by Immunohistochemistry.
  • Overlay histogram showing K562 cells stained with ab69856 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab69856, 0.5µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed. This antibody gave a positive signal in K562 cells fixed with 4% paraformaldehyde (10 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.

Anti-PAX3 antibody [C2] (ab69856) 使用論文

This product has been referenced in:
  • Hu Q  et al. Structural and functional studies of FKHR-PAX3, a reciprocal fusion gene of the t(2;13) chromosomal translocation in alveolar rhabdomyosarcoma. PLoS One 8:e68065 (2013). Read more (PubMed: 23799156) »

See 1 Publication for this product

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