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Rabbit Anti-PHOX2B/BF594 Conjugated antibody (bs-11578R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11578R-BF594
英文名稱 Rabbit Anti-PHOX2B/BF594 Conjugated antibody
中文名稱 BF594標記的神經母細胞瘤蛋白PHOX2B抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 神經生物學  信號轉導  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PHOX2B (101-200aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.
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