RHOT1 GTPase cycle

This pathway catalogues guanine nucleotide exchange factors (GEFs) and effectors of RHOT1 (also known as MIRO-1). RHOT1 possesses a high intrinsic GTP-ase activity and does not require a GTPase activator protein (GAP). No GDP dissociation inhibitors (GDIs) have been reported to interact with RHOT1. RHOT1 is a mitochondrial RHO GTPase. Like related RHOT2 (MIRO-2), RHOT1 localizes to the outer mitochondrial membrane. RHOT1 is implicated in mitochondrial movement inside the cells, including the axonal transport of mitochondria in neurons, as well as mitochondrial fission and fusion. RHOT1/RHOT2-mediated mitochondrial turnover is affected in neurodegenerative diseases. RHOT1 can localize to peroxisomes and regulate peroxisome motility and fission. RHOT1 is also involved in the regulation of ER-mitochondria membrane contact sites.

Summary from Reactome (R-HSA-9013425), read 2026-09-23.