Validation of blue- and clear-native polyacrylamide gel electrophoresis protocols to characterize mitochondrial oxidative phosphorylation complexes.

ABSTRACT: The mitochondrial oxidative phosphorylation (OXPHOS) system plays a pivotal role in the cell's energy conversion. The enzymes involved in OXPHOS are arranged in five protein-lipid complexes. The first four complexes (I-IV) form the mitochondrial respiratory chain, while Complex V is an F1Fo-ATP synthase. Mutations in genes involved in the biosynthesis of the OXPHOS complexes are an important cause of metabolic diseases. Blue-native polyacrylamide gel electrophoresis (BN-PAGE), originally developed by Hermann Schägger in the 1990s, has become instrumental in gaining insights into structure/function relationships of the OXPHOS system, including: (1) the assembly pathways of the complexes, (2) the composition of higher-order respiratory chain supercomplexes and (3) pathologic mechanisms in pa

SUBMITTER: Aref J 

PROVIDER: S-EPMC12445495 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature