ARHGEF10 is a guanine nucleotide exchange factor (GEF) that primarily activates small GTPases, particularly RhoB in endothelial cells and RhoA in other contexts. In developmental biology, ARHGEF10 is expressed in the midbrain, hindbrain, and hematopoietic system during early zebrafish embryogenesis 1 and plays a role in peripheral nerve myelination. Mechanistically, ARHGEF10 functions in membrane trafficking by interacting with Rab6A and Rab8A at exocytotic vesicles, connecting Rab signaling with RhoA during vesicular transport 2. In inflammatory contexts, TNF-induced ARHGEF10 selectively catalyzes RhoB activation to disrupt endothelial tight junctions and capillary barrier function 3. Disease relevance is significant: ARHGEF10 exhibits haploinsufficiency, with heterozygous deletions causing neurodevelopmental impairment including developmental delay, language/speech deficits, and motor impairment 4. The gene is implicated in neuromuscular diseases presenting with slowed nerve conduction velocity. Additionally, ARHGEF10 polymorphisms (rs2280887, rs9657362, rs4480162) are associated with increased ischemic stroke risk in Han Chinese populations 5, and a common variant (rs2280885) alters delta-6 desaturase activity, influencing triglyceride levels and hypertriglyceridemia susceptibility 6. Clinically, ARHGEF10 serves as a potential diagnostic biomarker for postmenopausal osteoporosis and stroke risk stratification.