Anti-FKRP antibody,Abcam,AB220059

Host

Rabbit

Reactivity

Human

Application

ICC/IF

Platform ID

BAB870427725

Abcam

Headquarters

Discovery Drive Cambridge Biomedical Campus Cambridge CB2 0AX UK

Abcam

Contact

Tel: +44 (0)1223 696000
Fax: +44 (0)1223 215 215

Email:

[email protected]

Product Specifications
Scientific Background

Specifications

NameAnti-FKRP antibody
Cat. No.AB220059
HostRabbit
IsotypeIgG
ReactivityHuman
ApplicationICC/IF
ClonalityPolyclonal
Concentration0.08 - 0.1 mg/mL Batch dependent concentration
ImmunogenRecombinant Fragment Protein within Human FKRP aa 300-450. The exact immunogen used to generate this antibody is proprietary information.
PurityAffinity purification Immunogen
Appearance/FormLiquid
ShippingBlue Ice
FormulationpH: 7.2 Preservative: 0.02% Sodium azide Constituents: PBS, 40% Glycerol (glycerin, glycerine)
Storage-20°C
Regulatory StatusResearch Use Only

Scientific Background

Target data 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 : 27194101, PubMed : 29477842, PubMed : 31949166). 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 : 27194101, PubMed : 29477842, PubMed : 31949166). See full target information FKRP

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