FITC标记的先天性眼外肌纤维化相关蛋白FEOM2抗体
产品名称： FITC标记的先天性眼外肌纤维化相关蛋白FEOM2抗体
英文名称： Anti-PHOX2A/FITC
产品编号： HZ-11577R-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-PHOX2A/FITC Conjugated antibody
FITC标记的先天性眼外肌纤维化相关蛋白FEOM2抗体
| 产品编号 | bs-11577R-FITC |
| 英文名称 | Anti-PHOX2A/FITC |
| 中文名称 | FITC标记的先天性眼外肌纤维化相关蛋白FEOM2抗体 |
| 别 名 | Aristaless homeobox (Drosophila) fibrosis of extraocular muscles congenital 2 autosomal recessive; Aristaless homeobox gene homolog (Drosophila); Aristaless homeobox homolog; Aristaless homeobox protein homolog; ARIX 1 homeodomain protein; ARIX; Arix homeodomain protein; ARIX1 homeodomain protein; CFEOM 2; CFEOM2; FEOM 2; FEOM2; Fibrosis of extraocular muscles congenital 2 autosomal recessive; MGC52227; NCAM 2; NCAM2; Paired like (aristaless) Homeobox 2A; Paired like homeobox 2a; Paired mesoderm homeobox 2a; Paired mesoderm homeobox protein 2A; Paired-like homeobox 2A; PHOX 2A; Phox2; Phox2a; PHX2A_HUMAN; Pmx 2a; Pmx2; Pmx2a. |
| 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 转录调节因子 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Cow, Horse, Rabbit, Sheep, |
| 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 | 30kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PHOX2A (41-140aa) |
| 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: The protein encoded by this gene contains a paired-like homeodomain most similar to that of the Drosophila aristaless gene product. The encoded protein plays a central role in development of the autonomic nervous system. It regulates the expression of tyrosine hydroxylase and dopamine beta-hydroxylase, two catecholaminergic biosynthetic enzymes essential for the differentiation and maintenance of the noradrenergic neurotransmitter phenotype. The encoded protein has also been shown to regulate transcription of the alpha3 nicotinic acetylcholine receptor gene. Mutations in this gene have been associated with autosomal recessive congenital fibrosis of the extraocular muscles. [provided by RefSeq, Jul 2008] Function: May be involved in regulating the specificity of expression of the catecholamine biosynthetic genes. Acts as a transcription activator/factor. Could maintain the noradrenergic phenotype. Subcellular Location: Nucleus. DISEASE: Defects in PHOX2A are the cause of congenital fibrosis of extraocular muscles type 2 (CFEOM2) [MIM:602078]. CFEOM encompasses several different inherited strabismus syndromes characterized by congenital restrictive ophthalmoplegia affecting extraocular muscles innervated by the oculomotor and/or trochlear nerves. CFEOM is characterized clinically by anchoring of the eyes in downward gaze, ptosis, and backward tilt of the head. CFEOM2 may result from the aberrant development of the oculomotor (nIII), trochlear (nIV) and abducens (nVI) cranial nerve nuclei. Similarity: Belongs to the paired homeobox family. Contains 1 homeobox DNA-binding domain. Database links: Entrez Gene: 401 Human Entrez Gene: 11859 Mouse Entrez Gene: 116648 Rat Omim: 602753 Human SwissProt: O14813 Human SwissProt: Q62066 Mouse SwissProt: Q62782 Rat Unigene: 705937 Human Unigene: 5028 Mouse Unigene: 2858 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
由该基因编码的蛋白质含有与果蝇无缘基因产物最相似的成对的同源结构域。编码的蛋白在自主神经系统的发育中起着核心作用。它调节酪氨酸羟化酶和多巴胺β羟化酶的表达，两种儿茶酚胺生物合成酶对分化和维持去甲肾上腺素能神经递质表型至关重要。编码的蛋白质也被证明调节α3烟碱型乙酰胆碱受体基因的转录。该基因突变与常染色体隐性遗传先天性眼外肌纤维化有关。