Accessory subunit NDUFB4 participates in mitochondrial complex I supercomplex formation.

ABSTRACT: Mitochondrial electron transport chain complexes organize into supramolecular structures called respiratory supercomplexes (SCs). The role of respiratory SCs remains largely unconfirmed despite evidence supporting their necessity for mitochondrial respiratory function. The mechanisms underlying the formation of the I₁III₂IV₁ "respirasome" SC are also not fully understood, further limiting insights into these processes in physiology and diseases, including neurodegeneration and metabolic syndromes. NDUFB4 is a complex I accessory subunit that contains residues that interact with the subunit UQCRC1 from complex III, suggesting that NDUFB4 is integral for I₁III₂IV₁ respirasome integrity. Here, we introduced specific point mutat

SUBMITTER: Parmar G 

PROVIDER: S-EPMC10862015 | biostudies-literature | 2024 Feb

REPOSITORIES: biostudies-literature