| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | NBLST2; NBPhox; PHOX 2B; PHOX2B; PMX 2B; PMX2B;;PHOX2B |
| WB Predicted band size | Calculated MW: 32 kDa ; Observed MW: 35 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse |
| Immunogen | A synthesized peptide derived from human PHOX2B |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于PHOX2B抗体的参考文献示例(内容为模拟，仅供参考)：
1. **文献名称**：*PHOX2B mutations and autonomic nervous system dysfunction in congenital central hypoventilation syndrome*
**作者**：Weese-Mayer, D.E. 等
**摘要**：研究利用PHOX2B抗体检测先天性中枢通气不足综合征(CCHS)患者中的PHOX2B蛋白表达异常，揭示其与自主神经系统发育缺陷的关联。
2. **文献名称**：*PHOX2B as a biomarker for neuroblastoma diagnosis and prognosis*
**作者**：Grimmer, M.R. 等
**摘要**：通过PHOX2B抗体免疫组化分析，证实PHOX2B在神经母细胞瘤中的高特异性表达，提出其作为临床诊断和预后评估的分子标记。
3. **文献名称**：*Functional analysis of PHOX2B gene mutations using antibody-based assays*
**作者**：Berry-Kravis, E.M. 等
**摘要**：结合PHOX2B抗体与基因测序技术，揭示PHOX2B突变导致蛋白质构象改变，影响其在神经嵴细胞分化中的功能。
4. **文献名称**：*Developmental expression of PHOX2B in the vertebrate nervous system*
**作者**：Brunet, J.F. 和 Pattyn, A.
**摘要**：利用PHOX2B抗体追踪胚胎发育过程中PHOX2B在自主神经节和脑干核团中的动态表达模式，解析其调控神经发育的机制。
(注：上述文献为示例，实际引用需以真实出版物为准。)
The PHOX2B antibody is a critical tool in neuroscience and oncology research, targeting the protein product of the PHOX2B gene, a transcription factor essential for autonomic nervous system development. PHOX2B encodes a homeodomain-containing protein that regulates the differentiation and survival of neurons in neural crest-derived structures, including sympathetic ganglia, parasympathetic neurons, and enteric nervous system precursors. Its expression is tightly linked to the development of noradrenergic neurons and the establishment of respiratory control circuits.
Clinically, PHOX2B is a biomarker for neuroblastoma and congenital central hypoventilation syndrome (CCHS). Germline mutations in PHOX2B, particularly polyalanine repeat expansions or frameshift variants, are causative in ~90% of CCHS cases, a disorder characterized by impaired autonomic control of breathing. In neuroblastoma, PHOX2B immunohistochemistry aids in diagnosing poorly differentiated tumors, as its nuclear staining is highly specific (∼95%) and sensitive (∼97%) for this malignancy. The antibody helps distinguish neuroblastoma from histological mimics and correlates with clinical subtypes, such as tumors associated with the *PHOX2B*-mutated Hirschsprung’s disease-CCHS-neuroblastoma triad.
Research applications include studying neural crest migration, autonomic dysfunction, and genetic pathways in neurocristopathies. The antibody’s utility in both diagnostic pathology and developmental biology underscores its importance in bridging basic science and clinical practice.
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