FITC标记的神经母细胞瘤蛋白PHOX2B抗体
产品名称： FITC标记的神经母细胞瘤蛋白PHOX2B抗体
英文名称： Anti-PHOX2B/FITC
产品编号： HZ-11578R-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： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-PHOX2B/FITC Conjugated antibody
FITC标记的神经母细胞瘤蛋白PHOX2B抗体
| 产品编号 | bs-11578R-FITC |
| 英文名称 | Anti-PHOX2B/FITC |
| 中文名称 | FITC标记的神经母细胞瘤蛋白PHOX2B抗体 |
| 别 名 | NBPHOX; Neuroblastoma paired type homeobox protein; Neuroblastoma Phox; Paired like homeobox 2b; Paired mesoderm homeobox protein 2B; Paired-like homeobox 2B; PHOX 2B; PHOX 2B homeodomain protein; PHOX2B; PHOX2B homeodomain protein; PHX2B_HUMAN; PMX 2B; PMX2B. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 神经生物学 信号转导 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, |
| 产品应用 | ICC=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 | 32kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PHOX2B (101-200aa) |
| 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: Phox2a (also designated Arix1) and Phox2b are closely related, paired-homeodomain transcription factors that are necessary for neuronal differentiation throughout the developing sympathetic, parasympathetic and enteric ganglia. All enteric nervous system cells evolve from the neural crest, and all cells that are undifferentiated initially express Phox2b. The cells that begin to differentiate along a neuronal lineage continue to express Phox2b, and begin to express Phox2a. Phox2b is required for the differentiation of all central and nonperipheral noradrenergic centers in the brain. In contrast, Phox2a controls only the differentiation of the main noradrenergic center of the brain, the locus coeruleus. Both Phox2a and Phox2b are crucial for the regulation of endogenous tyrosine hydroxylase and dopamine-beta hydroxylase, which are transiently expressed in neural crest cells. In addition, Phox2 proteins are sufficient to promote sympathetic neuron generation. The gene which encodes Phox2a maps to human chromosome 11q13.3-q13.4. Function: Involved in the development of several major noradrenergic neuron populations, including the locus coeruleus. Transcription factor which could determine a neurotransmitter phenotype in vertebrates. Enhances second-messenger-mediated activation of the dopamine beta-hydrolase and c-fos promoters, and of several enhancers including cAMP-response element and serum-response element. Subunit: Interacts with TRIM11 Subcellular Location: Nucleus. Tissue Specificity: Expressed in neuroblastoma, brain and adrenal gland. DISEASE: Defects in PHOX2B are a cause of congenital central hypoventilation syndrome (CCHS) [MIM:209880]; also known as congenital failure of autonomic control or Ondine curse. Most mutations consist of 5-10 alanine expansions in the poly-Ala region from amino acids 241-260. CCHS is a rare disorder characterized by abnormal control of respiration in the absence of neuromuscular or lung disease, or an identifiable brain stem lesion. A deficiency in autonomic control of respiration results in inadequate or negligible ventilatory and arousal responses to hypercapnia and hypoxemia. CCHS is frequently complicated with neurocristopathies such as Hirschsprung disease that occurs in about 16% of CCHS cases. Defects in PHOX2B are the cause of susceptibility to neuroblastoma type 2 (NBLST2) [MIM:613013]. A common neoplasm of early childhood arising from embryonic cells that form the primitive neural crest and give rise to the adrenal medulla and the sympathetic nervous system. Similarity: Belongs to the paired homeobox family. Contains 1 homeobox DNA-binding domain. Database links: Entrez Gene: 8929 Human Entrez Gene: 18935 Mouse Omim: 603851 Human SwissProt: Q99453 Human SwissProt: O35690 Mouse Unigene: 87202 Human Unigene: 62505 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
PHOX2A（也指定ARIX1）和PHOX2B是密切相关的，配对的同源结构域转录因子是整个分化的交感神经、副交感神经和肠神经节的神经元分化所必需的。所有的肠神经系统细胞从神经嵴开始进化，所有未分化的细胞最初表达PHOX2B，开始沿着神经元谱系分化的细胞继续表达PHOX2B，并开始表达PHOX2A。大脑中的中枢和非外周去甲肾上腺素能中心。相反，PHOX2A仅控制脑的主要去甲肾上腺素能中枢的分化，蓝斑。PHOX2A和PHOX2B对调节神经嵴细胞瞬时表达的内源性酪氨酸羟化酶和多巴胺β-羟化酶是至关重要的。此外，PHOX2蛋白足以促进交感神经元的产生。编码PHOX2A的基因定位于人类染色体11q133-q134。