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>   home   >   Products   >   Primary Antibodies   >   Signal Transduction   >   FKRP Antibody (C-term)   

FKRP Antibody (C-term)

Mouse Monoclonal Antibody (Mab)

  • WB - FKRP Antibody (C-term) AM2033b
    FKRP Antibody (C-term) (Cat. #AM2033b) western blot analysis in HL-60 cell line lysates (35μg/lane).This demonstrates the FKRP antibody detected the FKRP protein (arrow).
Product Information
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
Primary Accession Q9H9S5
Other Accession Q8CG64, NP_001034974.1
Reactivity Human
Predicted Mouse
Host Mouse
Clonality Monoclonal
Isotype IgG1
Clone Names 386CT13.1.9
Calculated MW 54568 Da
Antigen Region 418-444 aa
Additional Information
Gene ID 79147
Other Names Fukutin-related protein, 2---, FKRP
Target/Specificity This FKRP antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 418-444 amino acids from the C-terminal region of human FKRP.
Dilution WB~~1:100
Format Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.
StorageMaintain 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.
PrecautionsFKRP Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Function Catalyzes the transfer of CDP-ribitol to ribitol 5- phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine- beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:25279699, PubMed:26923585, PubMed:29477842). This constitutes the second step in the formation of the ribose 5- phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:25279699, PubMed:26923585, PubMed:29477842).
Cellular Location Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma {ECO:0000250|UniProtKB:Q8CG64}. Rough endoplasmic reticulum. Cytoplasm {ECO:0000250|UniProtKB:Q8CG64}. Note=According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted (PubMed:19900540) Localization at the cell membrane may require the presence of dystroglycan (By similarity). At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes (PubMed:17554798, PubMed:21886772). In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum (PubMed:15213246) {ECO:0000250|UniProtKB:Q8CG64, ECO:0000269|PubMed:15213246, ECO:0000269|PubMed:17554798, ECO:0000269|PubMed:19900540, ECO:0000269|PubMed:21886772}
Tissue Location Expressed in the retina (at protein level) (PubMed:29416295). Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver, kidney, and pancreas (PubMed:11592034)
Research Areas
Citations (0)

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This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan. Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq].


Kawahara, G., et al. Hum. Mol. Genet. 19(4):623-633(2010)
Crowther-Swanepoel, D., et al. Nat. Genet. 42(2):132-136(2010)
Lu, P.J., et al. Biochim. Biophys. Acta 1802(2):253-258(2010)
Hanisch, F., et al. J. Neurol. 257(2):300-301(2010)
Bourteel, H., et al. J. Neurol. Neurosurg. Psychiatr. 80(12):1405-1408(2009)

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$ 325.00
$ 109.00
Cat# AM2033b
Availability: In Stock
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