Fig. 6.

The artificial transcription factors JZif1 and its prototype Jazz impact the neuromuscular junction (NMJ) by utrophin up-regulation.

The panels illustrate a schematic representation of the utrophin-associated protein complex at mammalian neuromuscular junction (NMJ). Dystroglycan (DG) associates with rapsyn and rapsyn links Acetylcholine Receptors (AChRs) to the utrophin associated protein complex. Utrophin links the entire complex to the F-actin cytoskeleton. The artificial transcription factors JZif1 and Jazz bind the utrophin promoter ‘A’ at the 9-base pair long target sequence, thereby enhancing transcription of utrophin gene. The expression of JZif1 (and Jazz) gene improves the morphology/plasticity of neuromuscular junction.