Mitochondrial Rho GTPase 1 (MIRO1) is an enzyme that in humans is encoded by the RHOT1 gene on chromosome 17. The Ras homolog family member T1 (RHOT) is an atypical Rho Ca2+-binding GTPase that localizes to the mitochondria. RHOT1, the related protein RHOT2, the adaptor protein Milton, and the PTEN induced putative kinase 1 (PINK1), form a complex that is involved in axonal transport of mitochondria. Both PINK1 and Parkin target RHOT1 for phosphorylation and degradation, causing the arrest of mitochondrial motility.