Anti-NDUFA6 antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The NDUFA6 protein also known as NADH:Ubiquinone Oxidoreductase Subunit A6 is an essential component of the mitochondrial respiratory chain complex I. Weighing approximately 15 kDa this protein forms a part of the inner mitochondrial membrane mainly expressed in tissues with high energy demands such as the heart brain and skeletal muscle. The protein aids in the initial steps of electron transfer from NADH to ubiquinone which is an important mechanical function in cellular respiration.

Biological function summary

NDUFA6 participates in the assembly and stability of complex I which is the largest of the five complexes in the mitochondrial electron transport chain. By contributing to complex I NDUFA6 plays a pivotal role in ATP production through oxidative phosphorylation. It ensures the effective transfer of electrons and maintains the proton gradient necessary for ATP synthesis. The integration of NDUFA6 within complex I illustrates its biological necessity for proper cellular energy metabolism.

Pathways

NDUFA6 significantly impacts the oxidative phosphorylation pathway and mitochondrial complex I assembly pathway. Its interaction within these pathways influences the overall energy metabolism and cellular respiration efficiency. The protein engages with several partners including NDUFS2 and NDUFV1 which are also integral subunits of complex I. These interactions highlight how NDUFA6 tightly coordinates with other proteins to ensure optimal energy production.

Associated diseases and disorders

Defects in NDUFA6 have been associated with mitochondrial diseases such as Leber Hereditary Optic Neuropathy and mitochondrial complex I deficiency. These conditions lead to impaired energy metabolism affecting tissues with high energy demands. In these diseases interactions with proteins like NDUFS1 and other complex I subunits become altered disrupting normal mitochondrial function. Understanding these connections emphasizes the importance of NDUFA6 in maintaining mitochondrial health and cellular energy balance.