SPG7 encodes paraplegin, a catalytic component of the mitochondrial m-AAA protease complex essential for proteostasis of inner mitochondrial membrane proteins and neuronal development 1. The protein possesses both ATPase and protease activities, functioning in protein quality control by degrading misfolded polypeptides and mediating processing of specific regulatory proteins. SPG7 regulates mitochondrial calcium homeostasis by controlling degradation of calcium transport regulators SMDT1/EMRE and MCU, limiting calcium uptake capacity. The protein also plays a role in mitochondrial permeability transition pore (mPTP) regulation, where under normal conditions SPG7 is tethered to PHB1 to inhibit mPTP opening, but PHB1 downregulation enhances SPG7-AFG3L2 interaction leading to mPTP activation and inflammatory responses 2. Mutations in SPG7 cause hereditary spastic paraplegia type 7, an autosomal recessive neurodegenerative disorder characterized by progressive spasticity and weakness of lower extremities 3. SPG7 is among the most frequently mutated genes in hereditary spastic paraplegia, representing 4.8% of cases 3. Patients may present with additional movement disorders including parkinsonism, dystonia, tremor, and ataxia, particularly in complicated forms 4.