Rho GTPases play a fundamental role in numerous cellular processes that are initiated by extracellular stimuli that work through G protein coupled receptors. The encoded protein may form complex with G proteins and stimulate Rho-dependent signals. ARHGEF10 (rho guanine nucleotide exchange factor 10), also known as GEF10 or KIAA0294, is a 1,369 amino acid protein that contains one DBL-homology domain and is thought to play a role in myelination of peripheral nerves, specifically during development. ARHGEF10 is expressed as four alternatively spliced isoforms that are present at low levels in ovary, lung, testis and kidney, with considerably higher expression in the central and peripheral nervous systems.