Abcam, ab65243, Anti-FKRP antibody

Size: 100µg
Rabbit Polyclonal FKRP antibody. Suitable for WB and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Synthetic Peptide within Human FKRP.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Synthetic Peptide within Human FKRP. The exact immunogen used to generate this antibody is proprietary information.Q9H9S5

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.4Preservative: 0.02% Sodium azideConstituents: PBS, 50% Glycerol (glycerin, glycerine), 0.87% Sodium chloride, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Storage information-Stable for 12 months at -20°C

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
The FKRP protein short for fukutin-related protein plays an important role in the glycosylation of alpha-dystroglycan an important component of muscle tissue structure. FKRP weighs approximately 55 kDa and is present in several tissues with high expression levels found in skeletal and cardiac muscle. It resides mainly within the Golgi apparatus where it participates in the modification of glycoproteins.
Biological function summary
FKRP functions as a glycosyltransferase involved in the synthesis of O-linked mannose structures on alpha-dystroglycan. Alpha-dystroglycan forms part of the dystrophin-glycoprotein complex a critical assembly in muscle tissue for linking the extracellular matrix to the cytoskeleton. This connection facilitates cellular stability during muscle contraction and maintains tissue integrity.
Pathways
FKRP operates predominantly within the alpha-dystroglycan glycosylation pathway. This pathway ensures proper glycan structures are present on alpha-dystroglycan permitting its interaction with extracellular matrix components like laminin. FKRP's activity closely interacts with other proteins like fukutin and LARGE1 which also participate in the modification of alpha-dystroglycan therefore impacting overall cell-matrix interactions.
FKRP mutations can lead to muscular dystrophies such as Walker-Warburg syndrome and limb-girdle muscular dystrophy 2I. These disorders often result from deficient glycosylation of alpha-dystroglycan leading to compromised muscle function and stability. Variations in FKRP expression or function affect the dystrophin-glycoprotein complex and consequently influence the muscle tissue's ability to withstand mechanical stress implicating FKRP as a critical player in maintaining muscular health.