PAX9 (D9F1N) Rabbit mAb

• 产品编号：CST-12847S      品牌：CST       原厂货号：12847S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

PAX9 (D9F1N) Rabbit mAb兔单抗可以识别内源性的总PAX9蛋白。单克隆抗体由合成肽段免疫动物产生，该肽段与人PAX9蛋白的Asp141邻近氨基酸残基序列一致。Paired box (PAX)蛋白是一组在动物发育过程中发挥了重要的及多样性作用的转录因子家族（1）。人和其他哺乳动物中发现了9个PAX蛋白（PAX1-9）。它们都含有氨基端“配对”结构域，包括了2个具有DNA结合活性的螺旋-转角-螺旋基序（2）。PAX蛋白，基于一个羧基端同源域和中心八肽区域的存在与否可以根据不同的结构分为4个不同的亚型(I-IV)。亚型I (PAX1 和9)包括八肽但是缺乏同源域；亚型II (PAX2, 5, 和 8)包括了八肽和被截短的同源域；亚型III (PAX3 和7)包括了八肽和完整的同源域；和亚型IV (PAX4 和6)包括了一个完整的同源域但是缺乏八肽结构（2）。PAX蛋白在发育过程中发挥了重要作用调控了胚胎模式和器官发生过程中的转录调控网络（3）；PAX蛋白的一组亚型还在产后发育过程中发挥重要功能（4）。研究提示基因突变引发的PAX基因表达异常与多种癌症相关（1-3），部分亚型II和III被认为是癌症进程中可能的介导因子（2）。PAX9表达在咽弓间质(5,6)，对牙和其他咽弓衍生物的胚胎发育至关重要(5,7)。敲除PAX9的小鼠会缺少咽囊结构(例如胸腺，甲状旁腺)(5)，人体PAX9突变经常会导致先天性延迟发育不全综合症例如多数牙先天缺失和个别牙先天缺失(8,9)。PAX9可以和其他转录因子结合(例如MSX1)一调控BMP家族蛋白的表达(例如BMP4)以协调咽弓发育(10,11)。研究指出PAX9基因簇的扩增导致的PAX9的表达上调与肺癌的发生率增加相关(12)。

1. Lang, D. et al. (2007) Biochem Pharmacol 73, 1-14.
2. Robson, E.J. et al. (2006) Nat Rev Cancer 6, 52-62.
3. Wang, Q. et al. (2008) J Cell Mol Med 12, 2281-94.
4. Blake, J.A. et al. (2008) Dev Dyn 237, 2791-803.
5. Peters, H. et al. (1998) Genes Dev 12, 2735-47.
6. Peters, H. et al. (1998) Eur J Oral Sci 106 Suppl 1, 38-43.
7. Hetzer-Egger, C. et al. (2002) Eur J Immunol 32, 1175-81.
8. Bergendal, B. et al. (2011) Am J Med Genet A 155A, 1616-22.
9. Suda, N. et al. (2011) J Dent Res 90, 382-6.
10. Nakatomi, M. et al. (2010) Dev Biol 340, 438-49.
11. Wang, Y. et al. (2011) J Dent Res 90, 311-6.
12. Kendall, J. et al. (2007) Proc Natl Acad Sci U S A 104, 16663-8.

原厂资料：

Homology

Species predicted to react based on 100% sequence homology: Monkey

Specificity / Sensitivity

PAX9 (D9F1N) Rabbit mAb recognizes endogenous levels of total PAX9 protein.

PAX9 (D9F1N) Rabbit mAb兔单抗可以识别内源性的总PAX9蛋白。

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Asp141 of human PAX9 protein.

Background

Paired box (PAX) proteins are a family of transcription factors that play important and diverse roles in animal development (1). Nine PAX proteins (PAX1-9) have been described in humans and other mammals. They are defined by the presence of an amino-terminal "paired" domain, consisting of two helix-turn-helix motifs, with DNA binding activity (2). PAX proteins are classified into four structurally distinct subgroups (I-IV) based on the absence or presence of a carboxy-terminal homeodomain and a central octapeptide region. Subgroup I (PAX1 and 9) contains the octapeptide but lacks the homeodomain; subgroup II (PAX2, 5, and 8) contains the octapeptide and a truncated homeodomain; subgroup III (PAX3 and 7) contains the octapeptide and a complete homeodomain; and subgroup IV (PAX4 and 6) contains a complete homeodomain but lacks the octapeptide region (2). PAX proteins play critically important roles in development by regulating transcriptional networks responsible for embryonic patterning and organogenesis (3); a subset of PAX proteins also maintain functional importance during postnatal development (4). Research studies have implicated genetic mutations that result in aberrant expression of PAX genes in a number of cancer subtypes (1-3), with members of subgroups II and III identified as potential mediators of tumor progression (2).PAX9 is expressed in pharyngeal arch mesenchyme (5,6), and is essential for embryonic development of the teeth and other pharyngeal arch derivatives (5,7). In mice, deletion of PAX9 results in an absence of structures derived from the pharyngeal pouches (e.g. thymus, parathyroid glands) (5), while in humans, PAX9 mutations are frequently associated with congenital tooth agenesis syndromes such as oligodontia and hypodontia (8,9). PAX9 appears to interact with other transcription factors (e.g. MSX1) to regulate the expression of BMP-family proteins (e.g. BMP4) that orchestrate pharyngeal arch development (10,11). Increased expression of PAX9, resulting from amplification at the PAX9 locus, has also been linked in research studies to an increased incidence of lung cancer (12).

1. Lang, D. et al. (2007) Biochem Pharmacol 73, 1-14.
2. Robson, E.J. et al. (2006) Nat Rev Cancer 6, 52-62.
3. Wang, Q. et al. (2008) J Cell Mol Med 12, 2281-94.
4. Blake, J.A. et al. (2008) Dev Dyn 237, 2791-803.
5. Peters, H. et al. (1998) Genes Dev 12, 2735-47.
6. Peters, H. et al. (1998) Eur J Oral Sci 106 Suppl 1, 38-43.
7. Hetzer-Egger, C. et al. (2002) Eur J Immunol 32, 1175-81.
8. Bergendal, B. et al. (2011) Am J Med Genet A 155A, 1616-22.
9. Suda, N. et al. (2011) J Dent Res 90, 382-6.
10. Nakatomi, M. et al. (2010) Dev Biol 340, 438-49.
11. Wang, Y. et al. (2011) J Dent Res 90, 311-6.
12. Kendall, J. et al. (2007) Proc Natl Acad Sci U S A 104, 16663-8.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150
mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02%
sodium azide. Store at –20°C. Do not aliquot the antibody