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Rabbit Anti-NOR1/FITC Conjugated antibody
background:
Nur77 (also designated NGFI-B), Nurr1 (Nur-related factor 1), and NOR-1 (neuron-derived orphan receptor 1) constitute the NGFI-B subfamily within the nuclear receptor superfamily. Ligands for these protein have not been identified, and, therefore, they are designated “orphan nuclear receptors”. Genes of the NGFI-B subfamily are classified as immediate-early genes, which are induced rapidly, but transiently, in response to a variety of stimuli. They have been implicated in cell proliferation, differentiation, and apoptosis. The human NOR-1 gene maps to chromosome 9q, and encodes a protein which is expressed in heart, skeletal muscle, thymus, and spleen as well as in brain, where it is developmentally regulated. There-fore, NOR-1 may be involved in regulating neural differentiation. The NOR-1 gene also undergoes chromosomal translocation with the EWS gene to produce a protein thought to affect pre-mRNA splicing.
Function:
Binds to the B1A response-element.
Subcellular Location:
Nucleus.
Tissue Specificity:
Isoform alpha is highly expressed in skeletal muscle. Isoform beta is highly expressed in skeletal muscle and low expressed in fetal brain and placenta.
DISEASE:
Defects in NR4A3 are a cause of Ewing sarcoma (ES) [MIM:612219]. A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors. Note=A chromosomal aberration involving NR4A3 is found in patients with Erwing sarcoma. Translocation t(9;22)(q22-31;q11-12) with EWSR1.
Note=A chromosomal aberration involving NR4A3 is a cause of a form of extraskeletal myxoid chondrosarcomas (EMC). Translocation t(9;17)(q22;q11) with TAF2N.
Similarity:
Belongs to the nuclear hormone receptor family. NR4 subfamily.
Contains 1 nuclear receptor DNA-binding domain.
Database links:
Entrez Gene: 8013 Human
Entrez Gene: 18124 Mouse
Entrez Gene: 397479 Pig
Entrez Gene: 58853 Rat
Omim: 600542 Human
SwissProt: Q92570 Human
SwissProt: Q9QZB6 Mouse
SwissProt: P51179 Rat
Unigene: 279522 Human
Unigene: 247261 Mouse
Unigene: 62694 Rat
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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