先天性眼外肌纤维化相关蛋白FEOM2抗体说明书 英文名称  Anti-PHOX2A/CFEOM2

中文名称  先天性眼外肌纤维化相关蛋白FEOM2抗体说明书 

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.

产品属性：

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Chicken, Cow, Horse, Rabbit, Sheep

产品类型   一抗  

研究领域    神经生物学 信号转导 转录调节因子 表观遗传学

蛋白分子量  predicted molecular weight: 30kDa 

性    状   Lyophilized or Liquid

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   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

先天性眼外肌纤维化相关蛋白FEOM2抗体说明书 产品应用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品介绍 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 ceruleus. 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 : 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.

Contains 1 homeobox DNA-binding domain.

Database links : UniProtKB/Swiss-Prot: O14813.2

实验流程：

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