M. Zeviani

COA8 is a COX10-binding protein involved in the early biogenesis of cytochrome c oxidase

This study identifies COA8 as a COX10-binding protein essential for COX10 stability and catalytic activity in human cytochrome c oxidase biogenesis.

Structural rather than catalytic role for mitochondrial respiratory chain supercomplexes

This study shows that mitochondrial respiratory chain supercomplexes stabilize individual complexes under suboptimal conditions but do not enhance catalytic activity.

Biogenesis of NDUFS3-less complex I indicates TMEM126A/OPA7 as an assembly factor of the ND4-module

Opa1 overexpression protects from early onset Mpv17-/--related mouse kidney disease

This study shows that moderate Opa1 overexpression in Mpv17-/- mice delays kidney disease progression by preserving mitochondrial structure and function.

Loss of COX4i1 leads to combined respiratory chain deficiency and impaired mitochondrial proteosynthesis

This study shows that loss of COX4i1 causes combined respiratory chain deficiency and impaired mitochondrial proteosynthesis by disrupting cI and cIV biogenesis.

Inhibition of proteasomal degradation rescues a pathogenic variant of mitochondrial Respiratory chain assembly 1 factor

This study shows that inhibiting proteasome degradation rescues mutant RESA1 protein function and complex IV activity in patient-derived fibroblasts.