Anti-FKRP Polyclonal Antibody for WB, ELISA - Q9H9S5

Core Product Specifications and Parameters

Biological Background: FKRP Function and Localization

• FKRP (Fukutin-related protein) is a ribitol 5-phosphate transferase that catalyzes the second step in forming the ribitol 5-phosphate tandem repeat on alpha-dystroglycan, a key modification for extracellular matrix binding (PubMed:25279699, PubMed:26923585, PubMed:27194101, PubMed:29477842). Related references: PMID:25279699 PMID:26923585 PMID:27194101 PMID:29477842
• It is essential for the functional glycosylation of alpha-dystroglycan, which mediates interactions with laminin and other extracellular proteins, providing mechanical stability to muscle and nervous tissues.
• Subcellular localization includes the Golgi apparatus membrane, with additional presence in the sarcolemma, rough endoplasmic reticulum, cytoplasm, and secreted forms.
• FKRP is expressed predominantly in skeletal muscle, placenta, and heart, and more weakly in brain, lung, liver, kidney, and pancreas (PubMed:11592034). It is also detected in the retina at the protein level (PubMed:29416295). Related references: PMID:11592034 PMID:29416295
• Mutations in FKRP are linked to a spectrum of dystroglycanopathies, including limb-girdle muscular dystrophy, congenital muscular dystrophy, and cardiomyopathy.
• The protein contains a transmembrane helix, a signal-anchor, and conserved metal-binding sites (zinc/magnesium), which are critical for its transferase activity.
• FKRP is a member of the ribitol 5-phosphate transferase family and plays a non-redundant role in the dystroglycan glycosylation pathway despite its name similarity to fukutin.

Experimental Guidance and Technical Tips

• The immunogen corresponds to residues 30–220 of human FKRP, spanning the N-terminal luminal domain; the resulting polyclonal antibody may detect multiple epitopes and recognize both native and denatured protein. Confirm specificity using knockout or knockdown models.
• For Western blot, a band of approximately 55 kDa is expected under reducing conditions. Consider using a high-resolution gel and mild detergents to resolve membrane-associated forms; validate in relevant tissues like skeletal muscle or placenta.
• For ELISA, the recombinant immunogen can serve as a positive control. Perform checkerboard titrations to optimize antibody and antigen concentrations.
• Cross-reactivity with rat has been noted, but sequence homology with other species should be examined if other models are used; validate in the appropriate sample system.
• Given FKRP’s presence in multiple subcellular compartments, subcellular fractionation or immunoprecipitation may enhance detection in low-abundance samples.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio supports diagnostic manufacturers and researchers in the muscular dystrophy and glycobiology fields by offering comprehensive raw-material solutions. This anti-FKRP antibody exemplifies our ability to supply validated polyclonal antibodies for targets involved in rare glycosylation pathways. We can also source matched antibody pairs, optimized monoclonal antibodies, recombinant proteins, and ancillary reagents to streamline your IVD assay development from concept to clinic.