Anti-FKRP antibody (1-50 aa) (STJ93080)
SPECIFICATIONS
ClonalityPolyclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
ImmunogenThe antiserum was produced against synthesized peptide derived from the human FKRP at the amino acid range 1-50
General Information
| Short Description | Rabbit polyclonal anti-Ribitol 5-phosphate transferase FKRP (1-50 aa) for use in WB, IF and ELISA in Human, Mouse and Rat samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB/IF/ELISA |
| Host | Rabbit |
| Reactivity | Human/Mouse/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 | 1 mg/mL |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Dilution Range | WB 1:500-1:2000IF 1:200-1:1000ELISA 1:20000 |
| Formulation | Liquid in PBS containing 50% Glycerol, 0.5% BSA and 0.02% Sodium Azide. |
| 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 | The antiserum was produced against synthesized peptide derived from the human FKRP at the amino acid range 1-50 |
| Immunogen Region | 1-50 aa |
| Specificity | FKRP Polyclonal Antibody detects endogenous levels of FKRP protein. |
Additional Info
| 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