Parkinson's disease mutant Miro1 causes mitochondrial dysfunction and dopaminergic neuron loss
Mitochondrial impairment has been showed to be a major driver of neurodegeneration in Parkinson’s disease.
RHOT1 gene encodes for mitochondrial GTPase protein, Miro1, is essential for mitochondrial homeostasis as it is involved in mitochondrial transport, mitophagy and mitochondrial calcium buffering.
Miro1 has been linked genetically and pathophysiologically to PD but the molecular mechanisms underlying neurodegeneration in PD is not well understood.
Using neuronal cell cultures and midbrain organoids from PD pateints, the researchers show that a mutation in Miro1 causes increased oxidative stress, disrupted mitochondrial bioenergetics and function and dopaminergic neuron loss, further supporting its role in Parkinson’s disease pathology.
https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awaf051/8003628
