| Host: |
Rabbit |
| Applications: |
WB |
| Reactivity: |
Rat |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: |
Rabbit polyclonal antibody anti-FKRP (30-220) is suitable for use in Western Blot research applications. |
| Clonality: |
Polyclonal |
| Conjugation: |
Unconjugated |
| Isotype: |
IgG |
| Formulation: |
PBS with 0.05% Proclin300, 50% Glycerol, pH7.3. |
| Purification: |
Affinity purification |
| Dilution Range: |
WB 1:100-1:500 |
| Storage Instruction: |
Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: |
FKRP |
| Gene ID: |
79147 |
| Uniprot ID: |
FKRP_HUMAN |
| Immunogen Region: |
30-220 |
| Immunogen: |
Recombinant fusion protein containing a sequence corresponding to amino acids 30-220 of human FKRP (NP_077277.1). |
| Immunogen Sequence: |
QPRNSRARGPRRASAAGPRV TVLVREFEAFDNAVPELVDS FLQQDPAQPVVVAADTLPYP PLALPRIPNVRLALLQPALD RPAAASRPETYVATEFVALV PDGARAEAPGLLERMVEALR AGSARLVAAPVATANPARCL ALNVSLREWTARYGAAPAAP RCDALDGDAVVLLRARDLFN LSAPLARPVGT |
| 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 |
| 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
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
