The gene FXR1 (fragile X mental retardation syndrome-related protein 1) is mapped to human chromosome 3q28. FXR1 belongs to fragile X related family. It is an autosomal paralog of fragile X mental retardation 1. FXR1 is a RNA binding protein and is localized in the cytoplasm. RT-PCR analysis showed FXR1 expression in brain, heart, kidney and testis.
Synonyms: Anti-Fragile X mental retardation syndrome-related protein 1; Anti-hFXR1p
Storage: -20C
Application: Anti-FXR1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: Fragile X mental retardation syndrome-related protein 1 (FXR1) plays an important role in development as FXR1 knockout mice die early during embryogenesis. FXR1 is important for proper development of the muscle tissue. Facioscapulohumeral muscular dystrophy myoblasts show abnormal expression of FXR1 which deregulates the proper metabolism of muscle specific mRNAs. Similarly, miR369-3 mediated association of argonaute 2 and FXR1 with AU-rich elements directs translational activation. FXR1 forms a complex with oncogenes, protein kinase C, iota and epithelial cell transforming-2, resulting in tumor progression and non-small cell lung cancer growth. Binding of plakophilins 1/3 with FXR1 is important for stability of specific mRNAs. FXR1 also interacts with tudor domain-containing protein-3 and Bcl-2-associated transcription factor-1. Glycogen synthase kinase 3ß phosphorylates and down-regulates FXR1, thereby regulating behavior dimensions related to mood disorders in humans.
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