catalog number :
MBS9206242
products full name :
PHOX2B Antibody (Center)
products short name :
[PHOX2B]
products name syn :
[Paired mesoderm homeobox protein 2B; Neuroblastoma Phox; NBPhox; PHOX2B homeodomain protein; Paired-like homeobox 2B; PHOX2B; PMX2B]
other names :
[paired mesoderm homeobox protein 2B; Paired mesoderm homeobox protein 2B; paired mesoderm homeobox protein 2B; paired-like homeobox 2b; Neuroblastoma Phox; NBPhox; PHOX2B homeodomain protein; Paired-like homeobox 2B]
products gene name :
[PHOX2B]
other gene names :
[PHOX2B; PHOX2B; PMX2B; NBLST2; NBPhox; PMX2B; NBPhox]
uniprot entry name :
PHX2B_HUMAN
reactivity :
Mouse (Predicted Reactivity: Human, Rat)
sequence positions :
[104-130]
specificity :
This PHOX2B antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 104-130 amino acids from the Central region of human PHOX2B.
purity :
Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
form :
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
concentration :
Vial Concentration: 0.5
storage stability :
Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
tested application :
Immunofluorescence (IF), ELISA (EIA), Western Blot (WB)
app notes :
IF~~1:10~50. WB~~1:1000
image1 heading :
Immunofluorescence (IF)
image2 heading :
Western Blot (WB)
other info1 :
Antigen Type: Synthetic Peptide
other info2 :
Antigen Source: HUMAN. 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. Cellular Location: Nucleus {ECO:0000255 PROSITE- ProRule:PRU00108}.
Tissue Location: Expressed in neuroblastoma, brain and adrenal gland.
products categories :
Neuroscience
products description :
The DNA-associated protein encoded by this gene is a. member of the paired family of homeobox proteins localized to the. nucleus. The protein functions as a transcription factor involved. in the development of several major noradrenergic neuron. populations and the determination of neurotransmitter phenotype. The gene product is linked to enhancement of second. messenger-mediated activation of the dopamine beta-hydroylase,. c-fos promoters and several enhancers, including cyclic. amp-response element and serum-response element. [provided by. RefSeq].
products references :
Rudzinski, E., et al. Pediatr. Dev. Pathol. 13(4):291-299(2010). Janoueix-Lerosey, I., et al. Oncogene 29(11):1566-1579(2010). Tu, E., et al. Hum. Pathol. 41(3):392-400(2010). Arai, H., et al. J. Hum. Genet. 55(1):4-7(2010). Dubreuil, V., et al. J. Neurosci. 29(47):14836-14846(2009)
ncbi acc num :
NP_003915.2
ncbi gb acc num :
NM_003924.3
ncbi pathways :
, Pathway (8929); , Pathway (8929)
ncbi summary :
The DNA-associated protein encoded by this gene is a member of the paired family of homeobox proteins localized to the nucleus. The protein functions as a transcription factor involved in the development of several major noradrenergic neuron populations and the determination of neurotransmitter phenotype. The gene product is linked to enhancement of second messenger-mediated activation of the dopamine beta-hydroylase, c-fos promoters and several enhancers, including cyclic amp-response element and serum-response element. [provided by RefSeq, Jul 2008]
uniprot summary :
PHOX2B: 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. Defects in PHOX2B are a cause of congenital central hypoventilation syndrome (CCHS); 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). 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. Belongs to the paired homeobox family. Protein type: Transcription regulation; DNA-binding. Chromosomal Location of Human Ortholog: 4p12. Cellular Component: nuclear chromatin. Biological Process: hindbrain tangential cell migration; autonomic nervous system development; transcription, DNA-dependent; neuron migration; neurological control of breathing; enteric nervous system development; rhombencephalic reticular formation development; negative regulation of cell proliferation; sympathetic nervous system development; cell differentiation in hindbrain; negative regulation of neuron differentiation; parasympathetic nervous system development; glial cell differentiation; regulation of gene expression; efferent axon development in a lateral line nerve; positive regulation of transcription from RNA polymerase II promoter; positive regulation of neuron differentiation; inner ear development. Disease: Neuroblastoma, Susceptibility To, 2; Central Hypoventilation Syndrome, Congenital