BOLA1 (bolA family member 1) is a mitochondrial iron-sulfur (Fe-S) cluster assembly factor that facilitates Fe-S cluster insertion into mitochondrial proteins. BOLA1 forms [2Fe-2S] cluster-bridged heterodimeric complexes with the monothiol glutaredoxin GLRX5 1, functioning as a redox-regulatory protein rather than a cluster trafficking factor 2. The BOLA1-GLRX5 complex coordinates reduced, Rieske-type [2Fe-2S] clusters with preferential formation over alternative complexes 1. BOLA1 protects cells against oxidative stress by maintaining mitochondrial thiol redox potential and preventing morphology aberrations induced by glutathione depletion 3. BOLA1 dysregulation associates with multiple diseases. In hepatocellular carcinoma, elevated BOLA1 expression correlates with worse pathological grades, advanced TNM stages, and reduced overall survival 4. Similarly, in kidney renal clear cell carcinoma, high BOLA1 expression with gene hypomethylation predicts shorter survival and reduced relapse-free survival 5. Notably, Mendelian randomization analysis identified BOLA1 as a secondary mitochondrial-related gene associated with hyperuricemia risk 6. BOLA1 thus serves as both a critical Fe-S biogenesis factor and an emerging prognostic biomarker in malignancy.