製品の概要

  • 製品名Anti-PAX3 antibody
    PAX3 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to PAX3
  • 特異性Reactive with PAX3. A customer Abreview from January 2011 demonstrates the development-specific expression.
  • アプリケーション適用あり: WB, ELISAmore details
  • 種交差性
    交差種: Mouse, Human
    交差が予測される動物種: Rat, Rabbit, Goat, Horse, Chicken, Guinea pig, Cow, Cat, Dog
  • 免疫原

    Synthetic peptide: KKAKHSIDGI LSERASAPQS DEGSDIDSEP DLPLKRKQRR SRTTFTAEQL, corresponding to amino acids 181-230 of Human PAX3

  • ポジティブ・コントロール
    • Fetal liver lysate

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab50193 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
WB Use a concentration of 2.5 µg/ml. Detects a band of approximately 42 kDa (predicted molecular weight: 53 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA 1/312500.

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 画像

  • Anti-PAX3 antibody (ab50193) at 2.5 µg/ml + Fetal liver lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 53 kDa
    Observed band size : 42 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-PAX3 antibody (ab50193) at 1/1000 dilution

    Lane 1 : Whole tissue lysate prepared from mouse cerebellum (post natal 4 days)
    Lane 2 : Whole tissue lysate prepared from mouse cerebellum (adult)
    Lane 3 : Whole tissue lysate prepared from mouse kidney (adult)

    Lysates/proteins at 20 µg per lane.

    Secondary
    HRP conjugated mouse monoclonal at 1/10000 dilution

    Predicted band size : 53 kDa

    Image courtesy of an anonymous Abreview.

    See Abreview

Anti-PAX3 antibody (ab50193) 使用論文

ab50193 has not yet been referenced specifically in any publications.

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Glad to hear that the replacement vial was successfull. I checked our stocks and we have 3 more vials of ab59271, lot GR54001-1 in stock. It is the only lot in stock right now but just in case, simply request the specific lot you are interested in purc...

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Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Mouse Tissue lysate - whole (cerebellum P4 (post-natal day 4) and adult and ki)
Loading amount 20 µg
Specification cerebellum P4 (post-natal day 4) and adult and ki
Gel Running Conditions Reduced Denaturing (12%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 20°C
Username

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投稿 Jan 31 2011

Application Western blot
Sample Mouse Cell lysate - nuclear (p19 cell lysate and primary muscle cell lysate)
Specification p19 cell lysate and primary muscle cell lysate
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5%
Username

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Verified customer

投稿 Jun 05 2007

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