Dedicator of cytokinesis protein 7 (DOCK7)

Dedicator of cytokinesis protein 7 (DOCK7) is an atypical guanine nucleotide exchange factor that activates Rac1 and Rac3 GTPases by facilitating GDP-GTP exchange, distinct from canonical GEFs by its DHR2 domain. It plays critical roles in neuronal development—including axon formation and polarization, radial glial cell fate, and signal transduction—and is essential for normal muscle fusion and regeneration via cytoskeletal rearrangement. Pathogenic variants of DOCK7 cause severe neurodevelopmental disorders such as developmental and epileptic encephalopathy 23, with manifestations including epilepsy, intellectual disability, muscle hypotonia, and cortical blindness. DOCK7 is viewed as a high-potential therapeutic target, especially for conditions with impaired Rac1 pathway activation or cytoskeletal dysfunction.

Other names

KIAA1771ZIR2Q96N67 (UniProt)Q00M63Q2PPY7Q45RE8Q45RE9Q5T1B9Q5T1C0Q6ZV32Q8TB82Q96NG6Q96NI0Q9C092

Small molecules could inhibit or modulate DOCK7’s GEF activity, blocking Rac1/Rac3 activation; PI3K pathway modulation may affect Dock7 recruitment and downstream effects.