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. Author manuscript; available in PMC: 2022 Mar 1.
Published in final edited form as: Trends Cell Biol. 2020 Dec 1;31(3):197–210. doi: 10.1016/j.tcb.2020.11.003

Figure 3: Available in vitro and in vivo models to study FKRP mutations.

Figure 3:

Biological models of fukutin related protein (FKRP)-associated dystroglycanopathies have been developed using zebrafish (danio rerio), mouse (mus musculus), and human cells (homo sapiens). Several zebrafish and mouse FKRP models recapitulate the large spectrum of diseases. The recent use of patient-specific induced pluripotent stem cells (iPSCs) to recapitulate in vitro disease phenotype allowed for the generation of LGMDR9 iPSC-derived EBs and cardiomyocytes, as well as MDC1 iPSC-derived-neurons.