FITC标记的配对盒基因3抗体
产品名称： FITC标记的配对盒基因3抗体
英文名称： Anti-PAX3/FITC
产品编号： HZ-1097R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： Flow-Cyt=1:50-200 IF=1:50-200
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Rabbit Anti-PAX3/FITC Conjugated antibody (bs-1097R-FITC)
FITC标记的配对盒基因3抗体
| 英文名称 | Anti-PAX3/FITC |
| 中文名称 | FITC标记的配对盒基因3抗体 |
| 别 名 | CDHS; HUP 2; HUP2; MGC120381; MGC120382; MGC120383; MGC120384; MGC134778; paired box homeotic gene 3; Paired box protein Pax 3; Paired box protein Pax3; paired domain gene 3; paired domain gene HuP2; PAX 3; PAX3/FKHR fusion gene; Waardenburg syndrome 1; WS 1; WS1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 发育生物学 信号转导 干细胞 转录调节因子 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, |
| 产品应用 | Flow-Cyt=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 53kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PAX3 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: This protein is a member of the paired box (PAX) family of transcription factors. Members of the PAX family typically contain a paired box domain and a paired-type homeodomain. These proteins play critical roles during fetal development. Mutations in paired box gene 3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma. The translocation t(2;13)(q35;q14), which represents a fusion between PAX3 and the forkhead gene, is a frequent finding in alveolar rhabdomyosarcoma. Alternative splicing results in transcripts encoding isoforms with different C-termini. Function: Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis. Subunit: Can bind to DNA as a homodimer or a heterodimer with PAX7. Interacts with PAXBP1; the interaction links PAX3 to a WDR5-containing histone methyltransferase complex. Interacts with DAXX. Subcellular Location: Nucleus. DISEASE: Waardenburg syndrome 1 (WS1) [MIM:193500]: WS1 is an autosomal dominant disorder characterized by non-progressive sensorineural deafness, pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma, and wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum). WS1 shows variable clinical expression and some affected individuals do not manifest hearing impairment or iris pigmentation disturbances. Dystopia canthorum is the most consistent sign and is found in 98% of the patients. Note=The disease is caused by mutations affecting the gene represented in this entry. Waardenburg syndrome 3 (WS3) [MIM:148820]: WS3 is an autosomal dominant disorder characterized by sensorineural deafness, pigmentary disturbances, dystopia canthorum and limb anomalies such as hypoplasia of the musculoskeletal system, flexion contractures, fusion of the carpal bones, syndactylies. Note=The disease is caused by mutations affecting the gene represented in this entry. Craniofacial-deafness-hand syndrome (CDHS) [MIM:122880]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Rhabdomyosarcoma 2 (RMS2) [MIM:268220]: A form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchymal 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=The gene represented in this entry is involved in disease pathogenesis. 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. Similarity: Belongs to the paired homeobox family. Contains 1 homeobox DNA-binding domain. Contains 1 paired domain. Database links: Entrez Gene: 5077 Human Entrez Gene: 18505 Mouse Entrez Gene: 114502 Rat Omim: 606597 Human SwissProt: P23760 Human SwissProt: P24610 Mouse SwissProt: Q0IH87 Xenopus laevis SwissProt: Q645N4 Xenopus laevis Unigene: 42146 Human Unigene: 1371 Mouse Unigene: 225252 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 同源结构域蛋白（Homeodomain Proteins） PAX-3属于转录抑制因子。在胚胎发育和肿瘤生长中起关键作用。其突变和某些肿瘤的发病有关 |
这种蛋白质是成对盒（PAX）转录因子家族的成员。PAX家族的成员通常包含成对的盒结构域和配对型同源结构域。这些蛋白在胎儿发育过程中起着关键性的作用。配对盒基因3的突变与Waardenburg综合征、颅面耳聋-手综合征和肺泡横纹肌肉瘤有关。肺泡型横纹肌肉瘤易位t(2;13)(q35;q14)是肺泡型横纹肌肉瘤的常见表现，它代表PAX3与叉头基因的融合。选择性剪接结果编码不同亚型的同型异构体。