ADCK2 is a mitochondrial aarF domain-containing kinase that plays a critical role in coenzyme Q (CoQ) biosynthesis and cellular bioenergetics 12. Primary function: ADCK2 regulates mitochondrial CoQ precursor import and is essential for maintaining mitochondrial respiratory capacity and fatty acid β-oxidation in skeletal muscle 2. Mechanism: ADCK2 haploinsufficiency reduces CoQ biosynthesis, impairing mitochondrial energy production and metabolic capacity 1. The protein operates through non-conventional signaling pathways, including RELB-dependent NFκB signaling and superoxide regulation 3. Disease relevance: ADCK2 deficiency causes adult-onset mitochondrial myopathy characterized by muscle weakness, lipid accumulation, and reduced physical performance 2. Notably, prenatal ADCK2 haploinsufficiency causes developmental delays, with defects detectable during embryonic development and progressive muscle wasting with aging 1. Clinical significance: ADCK2 is overexpressed in non-small cell lung cancer (NSCLC), correlating with poor survival and immunotherapy resistance; ADCK2 depletion suppresses cancer cell viability through Akt-mTOR pathway inactivation 4. CoQ10 supplementation provides partial therapeutic benefit for CoQ deficiency, with greatest efficacy when initiated prenatally or during development 1.