Mitochondrial Disease
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Question 1

A 2-year-old boy presents with progressive psychomotor regression following a febrile illness, nystagmus, and respiratory irregularities. MRI shows bilateral symmetric T2 hyperintensity in the caudate, putamen, and dorsal brainstem. Genetic testing reveals a homozygous pathogenic variant in SURF1. This gene encodes a protein involved in the assembly of which respiratory chain complex?