Anti-FKRP antibody (30-220) (STJ112232)
SPECIFICATIONS
ClonalityPolyclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
General Information
| Short Description | Rabbit polyclonal FKRP (30-220) antibody for use in WB and ELISA in rat samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB/ELISA |
| Host | Rabbit |
| Reactivity | Rat |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Clonality | Polyclonal |
| Isotype | IgG |
| Conjugation | Unconjugated |
| Concentration | Lot specific |
| Purification | Affinity purification |
| Dilution Range | WB:1:100-1:500ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Formulation | PBS with 0.09% Sodium Azide, 50% Glycerol, pH7.3. |
| Storage Instruction | Store at-20ยฐC for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Target Information
| Gene Symbol | FKRP |
| Gene ID | 79147 |
| Uniprot ID | FKRP_HUMAN |
| Immunogen Region | 30-220 |
| Immunogen Sequence | QPRNSRARGPRRASAAGPRV TVLVREFEAFDNAVPELVDS FLQQDPAQPVVVAADTLPYP PLALPRIPNVRLALLQPALD RPAAASRPETYVATEFVALV PDGARAEAPGLLERMVEALR AGSARLVAAPVATANPARCL ALNVSLREWTARYGAAPAAP RCDALDGDAVVLLRARDLFN LSAPLARPVGT |
| Specificity | Recombinant fusion protein containing a sequence corresponding to amino acids 30-220 of human FKRP (NP_077277.1). |
Additional Info
| Tissue Specificity | Expressed in the retina (at protein level). Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver, kidney, and pancreas. |
| Post Translational Modifications | N-glycosylated. |
| 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). 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. |
| Protein Name | Ribitol 5-Phosphate Transferase FkrpFukutin-Related ProteinRibitol-5-Phosphate Transferase |
| Database Links | |
| Cellular Localisation | Golgi Apparatus MembraneSingle-Pass Type Ii Membrane ProteinSecretedCell MembraneSarcolemmaRough Endoplasmic ReticulumCytoplasmAccording To Some Studies The N-Terminal Hydrophobic Domain Is Cleaved After Translocation To The Golgi Apparatus And The Protein Is SecretedLocalization At The Cell Membrane May Require The Presence Of DystroglycanAt The Golgi Apparatus Localizes To The Middle-To-Trans-CisternaeAs Assessed By Mg160 ColocalizationDetected In Rough Endoplasmic Reticulum In MyocytesIn GeneralMutants Associated With Severe Clinical Phenotypes Are Retained Within The Endoplasmic Reticulum |
| Alternative Antibody Names | Anti-Ribitol 5-Phosphate Transferase Fkrp antibodyAnti-Fukutin-Related Protein antibodyAnti-Ribitol-5-Phosphate Transferase antibodyAnti-FKRP antibody |
Information sourced from Uniprot.org