Fragile X Mental Retardation Protein 1 (FMR1, FMR-1, FMRP, FRAXA, MGC87458, POF, POF1, Protein FMR-1)

Fragile X Mental Retardation Protein (FMRP) is an RNA-Binding protein that is associated to polysomes and may be involved in the transpot of mRNA from the nucleus to the cytoplasm. Defects in FMR1 are the cause of Fragile X syndrome, which is a common genetic disease characterized by moderate to severe mental retardation, macroorchidism, large ears, prominent jaw, and high-pitched, jocular speech. The defect in most fragile X syndrome patients results from an amplication of a CGG repeat regions with is directly in front of the coding region.\n\nApplications: \nSuitable for use in Western Blot, ELISA, Immunohistochemistry, Immunocytochemistry and the detection of FMRP on blood smears. Other applications have not been tested.\n\nRecommended Dilutions:\nWestern Blot: 1:500 detected FMRP on 10ug of mouse E17 spinal cord lysate. Lysate was resolved by electrophoresis, transferred to PVDF membrane and probed with F6072. Proteins were visualized using an IgG (HRP) goat anti-mouse and a chemiluminescence detection system.\nELISA: 1:500-1:5000\nImmunohistochemistry (Paraffin and Frozen): 1:500-1:5000\nImmunocytochemistry: 1:500-1:5000 was used on transfected cells. Light fixation (2% PFA, permeabilize with 0.1% Triton in block only)\nDetection of FMRP on Blood Smears: 1:500-1:5000 \nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nHeLa whole cell lysatre, HeLa nuclear lysate, mouse E17 spinal cord lysate\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

F6072

Size

100ul

Applications

ELISA, ICC, IHC, WB

Hosts

Mouse

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid. No preservatives added.

P Type

Mab

Purity

Ascites

Isotype

IgG1,k

References

1. Nature Genetics 4: 335-340 (1993). 2. Lancet 345: 1147-1148 (1995). 3. Nature Genetics 10: 483-485 (1995). 4. Human Molecular Genetics 4: 783-789 (1995). 5. Lin, C.H., et al., J. Biochemistry 128: 493-498 (2000).

Additional Info

Recognizes human FMRP at ~71kD. The epitope is localized in the N-terminal half of FMRP. Crossreaction with FXR protein may be detected in cases of high expression of the latter proteins. \nSpecies Crossreactivity: mouse and rat