FKRP Antibody
(Synonyms: Ribitol 5-phosphate transferase FKRP, Fukutin-related protein, Ribitol-5-phosphate transferase, FKRP)FKRP Antibody is a Rabbit-derived and non-conjugated IgG Polyclonal antibody, targeting to FKRP.
-
Host:
Rabbit
-
Isotype:
IgG
-
Application:
WB, IHC-P
-
Reactivity :
Human, Mouse, Rat
-
Formulation:
Supplied in PBS (pH 7.4), containing 30% glycerol, and 0.01% sodium azide.
-
Conjugation:
Non-conjugated
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
Applications
| Application |
WB
WB: Western Blot
|
IHC-P
IHC-P: Immunohistochemistry-Paraffin
|
|---|---|---|
| Dilution Ratio | 1:1000-2000 | 1:100-200 |
Product Details
FKRP Antibody is a Rabbit-derived and non-conjugated IgG Polyclonal antibody, targeting to FKRP.
-
Host Rabbit
-
Clonality Polyclonal
-
Species ReactivityHuman, Mouse, Rat
-
Observed Molecular WeightObserved band size: 45 kDa
Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
-
Calculated Molecular Weight Predicted band size: 54 kDa
Purified recombinant protein of human FKRP
Endogenous
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
-
Appearance
Solution
-
Formulation
Supplied in PBS (pH 7.4), containing 30% glycerol, and 0.01% sodium azide.
-
Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
-
Shipping
Shipping with blue ice.
Background
-
Function
FKRP 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[1][2][3][4][5].
-
Subcellular Localization
Golgi apparatus membrane; Secreted; Cell membrane, sarcolemma; Rough endoplasmic reticulum; Cytoplasm
-
Expression
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. -
Isoforms & Post-Translational Modification
FKRP has an amino acid length of 495, molecular weight is 54568 Da.可发生 N-糖基化FKRP 的氨基酸个数为 495 个，分子量为 54568 Da。N-glycosylated
-
Subunit
Homodimer; disulfide-linked.
-
SwissProt ID
-
Synonyms
Ribitol 5-phosphate transferase FKRP, Fukutin-related protein, Ribitol-5-phosphate transferase, FKRP
Documentation
[1]. Kanagawa M, et al. Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy. Cell Rep. 2016 Mar 8;14(9):2209-2223. [Content Brief]
[2]. Gerin I, et al. ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto α-dystroglycan. Nat Commun. 2016 May 19;7:11534. [Content Brief]
[3]. Nishihara R, et al. Cell endogenous activities of fukutin and FKRP coexist with the ribitol xylosyltransferase, TMEM5. Biochem Biophys Res Commun. 2018 Mar 18;497(4):1025-1030. [Content Brief]
[4]. Kuwabara N, et al. Crystal structures of fukutin-related protein (FKRP), a ribitol-phosphate transferase related to muscular dystrophy. Nat Commun. 2020 Jan 16;11(1):303. [Content Brief]
[5]. Willer T, et al. The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylation. Elife. 2014 Oct 3;3:. [Content Brief]
Powered by Bioz
