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Item number | Size | Datasheet | Manual | SDS | Delivery time | Quantity | Price |
---|---|---|---|---|---|---|---|
ELK-ES7567.50 | 50 µl | - | - |
Request delivery time estimate |
169.00€
|
||
ELK-ES7567.100 | 100 µl | - | - |
Request delivery time estimate |
283.00€
|
If you have any questions, please use our Contact Form.
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for... more
Product information "Anti-FKRP"
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, Oct 2008], Protein function: Catalyzes the transfer of a ribitol 5-phosphate from CDP-L- ribitol to the ribitol 5-phosphate previously attached by FKTN/fukutin 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, PubMed:29477842, PubMed:31949166, PubMed:27194101). 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, PubMed:31949166, PubMed:27194101). [The UniProt Consortium] Recommended dilutions: Western Blot: 1/500 - 1/2000. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/20000. Not yet tested in other applications.. Cellular localization: Golgi apparatus membrane , Single-pass type II membrane protein . Secreted . Cell membrane, sarcolemma . Rough endoplasmic reticulum . Cytoplasm . 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). .
Keywords: | Anti-Fukutin-related protein, Anti-Ribitol-5-phosphate transferase, Anti-Ribitol 5-phosphate transferase FKRP, FKRP rabbit pAb |
Supplier: | ELK Biotechnology |
Supplier-Nr: | ES7567 |
Properties
Application: | WB, IF, ELISA |
Antibody Type: | Polyclonal |
Conjugate: | No |
Host: | Rabbit |
Species reactivity: | human, mouse, rat |
Immunogen: | The antiserum was produced against synthesized peptide derived from human FKRP. AA range:1-50 |
MW: | 50 kD |
Format: | Antiserum |
Database Information
KEGG ID : | K19873 | Matching products |
UniProt ID : | Q9H9S5 | Matching products |
Gene ID | GeneID 79147 | Matching products |
Handling & Safety
Storage: | -20°C |
Shipping: | +4°C (International: +4°C) |
Caution
Our products are for laboratory research use only: Not for administration to humans!
Our products are for laboratory research use only: Not for administration to humans!
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