Fragile X Mental Retardation, Autosomal Homolog 2 (FXR2) (AA 520-639) antibody
|Synonyms||FMR1L2, Fxr2h, MGC56215, zgc:56215, zgc:77472, id:ibd5046, FXR2|
Alternatives Western Blotting (WB), Immunofluorescence (IF)
|4 references available|
|Quantity||50 µg (250 µg/ml)|
|Price||Product not available in this region.|
The fragile X syndrome results from amplification of a polymorphic CGG repeat in the 5' untranslated region of the FMR1 gene and is the most common form of inherited mental retardation. Although the lack of expression of FMR1 protein produces the fragile X syndrome, the pathological mechanism is not known. FMR1 is an RNA binding protein that performs a chaperone or transporter function. It interacts with FXR1 and FXR2. These proteins have 60% identity with FMR1 and have similar KH and RNA binding domains. All three proteins are cytosolic, associate with the 60S ribosomal subunit, and form homomers or heteromers with each other. During embryonic development, FMR1, FXR1, and FXR2 are expressed primarily in the nervous system. Small amounts of FMR1, FXR1, and FXR2 shuttle between the cytoplasm and nucleus. FMR1 is found in the nucleoplasm, while FXR2 is localized to the nucleolus. Thus, FMR1, FXR1, and FXR2, either individually or in tandem, function to transport RNAs throughout the cytoplasm and to specific sites in the nucleus. Alterations in the interactions between these proteins and ribosomal components may underlie the pathology that produces fragile X syndrome.
Synonyms: Fragile X
1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Source of all serum proteins is from USDA inspected abattoirs located in the United States.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Please refer to us for technical protocols.
|Molecular Weight||95 kDa|
Related Products: ABIN968539, ABIN967389
|Purification||Purified from tissue culture supernatant or ascites by affinity chromatography.|
|Buffer||Aqueous buffered solution containing BSA, glycerol.|
|Preservative||0.09% Sodium azide.|
|Storage||Store undiluted at -20°C.|
|Research Area||Chromatin and Nuclear Signaling, DNA/RNA|
|Restrictions||For Research Use only|
Zhang, OConnor, Siomi et al.: "The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2." in: The EMBO journal, Vol. 14, Issue 21, pp. 5358-66, 1996 (PubMed).
Siomi, Zhang, Siomi et al.: "Specific sequences in the fragile X syndrome protein FMR1 and the FXR proteins mediate their binding to 60S ribosomal subunits and the interactions among them." in: Molecular and cellular biology, Vol. 16, Issue 7, pp. 3825-32, 1996 (PubMed).
Tamanini, Bontekoe, Bakker et al.: "Different targets for the fragile X-related proteins revealed by their distinct nuclear localizations." in: Human molecular genetics, Vol. 8, Issue 5, pp. 863-9, 1999 (PubMed).
Agulhon, Blanchet, Kobetz et al.: "Expression of FMR1, FXR1, and FXR2 genes in human prenatal tissues." in: Journal of neuropathology and experimental neurology, Vol. 58, Issue 8, pp. 867-80, 1999 (PubMed).
|Hosts||Rabbit (32), Mouse (11)|
|Reactivities||Human (41), Mouse (Murine) (26), Rat (Rattus) (23), Cow (Bovine) (20), Dog (Canine) (20), Chicken (19), Horse (Equine) (18), Pig (Porcine) (18), Rabbit (18), Cat (Feline) (1)|
|Applications||Western Blotting (WB) (28), Immunofluorescence (IF) (26), ELISA (20), Immunoprecipitation (IP) (8), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)) (7), Immunohistochemistry (Formalin-fixed Sections) (IHC (f)) (3), Immunohistochemistry (IHC) (3), Immunocytochemistry (ICC) (2), Immunoelectron Microscopy (IEM) (1)|
|Conjugates||Alexa Fluor 350 (1), Alexa Fluor 488 (1), Alexa Fluor 555 (1), Alexa Fluor 647 (1), Biotin (1), Cy3 (1), Cy5 (1), Cy5.5 (1), Cy7 (1), FITC (1), Gold (1), HRP (1), PE (1), PE-Cy3 (1), PE-Cy5 (1), PE-Cy5.5 (1), PE-Cy7 (1)|
|Epitopes||C-Term (2), AA 414-658 (1), Internal Region (1)|