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PHOX2A Rabbit pAb (bs-11577R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-11577R
英文名称 PHOX2A Rabbit pAb
中文名称 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.  
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
克 隆 号
交叉反应 (predicted: Human,Mouse,Rat,Rabbit,Sheep,Cow,Chicken,Horse)
产品应用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 30 kDa
检测分子量
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PHOX2A: 41-140/284 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
O14813

Gene ID:
401

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



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