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Fkrp antibody - C-terminal region
Rabbit Polyclonal Antibody
- 产品详情
- 实验流程
Application 
| WB |
|---|---|
| Primary Accession | Q8CG64 |
| Other Accession | NM_173430, NP_775606 |
| Reactivity | Human, Mouse, Rat, Rabbit, Zebrafish, Pig, Dog, Guinea Pig, Bovine |
| Predicted | Human, Mouse, Rat, Rabbit, Zebrafish, Pig, Bovine |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 54852 Da |
| Gene ID | 243853 |
|---|---|
| Alias Symbol | A830029B19Rik, AI842067, AI847300, LGMD1I, MDC1C |
| Other Names | Fukutin-related protein, 2.-.-.-, Fkrp |
| Format | Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. |
| Reconstitution & Storage | Add 50 ul of distilled water. Final anti-Fkrp antibody concentration is 1 mg/ml in PBS buffer with 2% sucrose. For longer periods of storage, store at 20°C. Avoid repeat freeze-thaw cycles. |
| Precautions | Fkrp antibody - C-terminal region is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | Fkrp {ECO:0000312|MGI:MGI:2447586} |
|---|---|
| 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:26923585). 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:26923585). |
| Cellular Location | Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum {ECO:0000250|UniProtKB:Q9H9S5}. Cytoplasm. Note=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 (PubMed:17452335). At the Golgi apparatus localizes to the middle-to-trans-cisternae (PubMed:12471058, PubMed:17554798). Detected in rough endoplasmic reticulum in myocytes (By similarity). {ECO:0000250|UniProtKB:Q9H9S5, ECO:0000269|PubMed:12471058, ECO:0000269|PubMed:17452335, ECO:0000269|PubMed:17554798, ECO:0000269|PubMed:19900540} |
| Tissue Location | Expressed in the retina, specifically in the inner segments of the photoreceptors, the outer plexiform layers, inner nuclear layers, and ganglion cell layers (at protein level) (PubMed:29416295). Expressed at highest levels in brain, lung, heart, kidney and liver (PubMed:12471058). |
Research Areas
For Research Use Only. Not For Use In Diagnostic Procedures.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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