概述

  • 产品名称Anti-PAX3抗体[6288D4a]
    参阅全部 PAX3 一抗
  • 描述
    小鼠单克隆抗体[6288D4a] to PAX3
  • 经测试应用适用于: WB, Dot blotmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant human PAX3

性能

应用

Our Abpromise guarantee covers the use of ab53571 in the following tested applications.

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

应用 Ab评论 说明
WB Use at an assay dependent dilution. Detects a band of approximately 40 kDa (predicted molecular weight: 53 kDa).
Dot blot Use at an assay dependent dilution.

靶标

  • 功能Probable transcription factor associated with development of alveolar rhabdomyosarcoma.
  • 疾病相关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.
  • 序列相似性Belongs to the paired homeobox family.
    Contains 1 homeobox DNA-binding domain.
    Contains 1 paired domain.
  • 细胞定位Nucleus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 [6288D4a] 图像

Anti-PAX3 antibody [6288D4a] (ab53571)参考文献

This product has been referenced in:
  • Li L  et al. Downregulation of microRNAs miR-1, -206 and -29 stabilizes PAX3 and CCND2 expression in rhabdomyosarcoma. Lab Invest 92:571-83 (2012). WB ; Human . Read more (PubMed: 22330340) »

See 1 Publication for this product

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