Human Dystroglycan Antibody

Detection of Human Dystroglycan by Western Blot

Hit compound 4BPPNit validation in the FKRP dystroglycanopathy human iPSC modelsFKRP‐NSCs treated with 4BPPNit showed increased IIH6 reactivity, compared with untreated control cells. Note that the augmented IIH6 reactivity by 4BPPNit is much weaker than that in CRISPR‐corrected‐NSCs. Under the same exposure time, the signal intensity of IIH6 reactivity is saturated in corrected‐NSCs.Representative immunoblots with the IIH6 antibody using FKRP‐ and corrected‐NSCs treated with 4BPPNit for 24, 48, and 72 h, and DMSO only controls.Quantification of alpha‐dystroglycan glycosylation (IIH6), compared with DMSO only controls.Representative immunoblots of laminin‐binding analysis using FKRP‐ and corrected‐NSCs treated with 4BPPNit and DMSO only controls.Quantification of laminin‐binding activities, compared with DMSO only controls.Representative immunoblots with the AF6868 antibody detecting both alpha‐dystroglycan core protein and beta‐dystroglycan in FKRP‐ and corrected‐NSCs treated with 4BPPNit and DMSO only controls.Quantification of alpha‐dystroglycan core protein and beta‐dystroglycan expression, compared with DMSO only controls.Data information: Intensities of glycosylation, laminin‐binding activity, or protein expression are normalized to GAPDH protein expression. Note that the left and right panels of the immunoblots in (B) and (D) are not equivalent exposure time. See also Appendix Fig S5. Values indicate mean ± s.d. (n = 3 or 4 biological replicates; one‐way ANOVA; NS, not significant; *P < 0.05; **P < 0.01). Image collected and cropped by CiteAb from the following publication (https://pubmed.ncbi.nlm.nih.gov/31566294), licensed under a CC-BY license. Not internally tested by R&D Systems.