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RHOT2 is a member of the Rho GTPase family and one of two isoforms of the protein

Miro: RHOT1 (Miro1) and RHOT2 (Miro2).[6][9] Compared to the rest of the Rho GTPase
family, the Miro isoforms are considered atypical due to their different
regulation.[10] Moreover, the Miro isoforms are only expressed in the mitochondria.
[11]

Miro associates with Milton (TRAK1/2) and the motor proteins kinesin and dynein to
form the mitochondrial motor/adaptor complex. Miro functions to tether the complex
to the mitochondrion while the complex transports the mitochondrion via
microtubules within cells.[6][7] Though Miro has been predominantly studied in
neurons, the protein has also been observed to participate in the transport of
mitochondria in lymphocytes toward inflamed endothelia.[9]

The motor/adaptor complex is regulated by calcium ion levels. At high


concentrations, calcium ions arrest mitochondrial transport by binding Miro,
causing the complex to detach from the organelle. Considering that physiological
factors such as activation of glutamate receptors in dendrites, action potentials
in axons, and neuromodulators may elevate calcium ion levels, this regulatory
mechanism likely serves to keep mitochondria in such areas to provide calcium ion
buffering and active export and, thus, maintain homeostasis.[6]

In addition, Miro regulates mitochondrial fusion and mitophagy in conjunction with


mitofusin. According to one model, damaged mitochondria are sequestered from
healthy mitochondria by the degradation of Miro and mitofusin. Miro degradation
halts their movement while mitofusin degradation prevents them from fusing with
healthy mitochondria, thus facilitating their clearance by autophagosomes. [6]

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