Recombinant Human NDUFAF4 protein (His tag N-Terminus)

Supplementary information

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

NDUFAF4 also known as Complex I Assembly Factor 2 plays an integral role in the assembly of NADH:ubiquinone oxidoreductase complex I within mitochondria. It has a mass of approximately 20 kilodaltons. This protein is widely expressed in various tissues with heightened levels found in organs with high energy demands such as the brain and heart. Functionally NDUFAF4 mediates the proper formation and stabilization of the mitochondrial complex I by contributing to the assembly of its subunits.

Biological function summary

NDUFAF4 participates in the mitochondrial electron transport chain as part of the complex I assembly. It acts as an assembly factor for complex I the first component of the chain which is important for initiating electron transport from NADH to the rest of the chain. This complex contains multiple subunits that require precise assembly and NDUFAF4 ensures the correct incorporation of these subunits. Its role is essential for maintaining efficient mitochondrial respiration thereby supporting ATP production.

Pathways

The NDUFAF4 protein ensures proper electron transport chain function by facilitating complex I assembly. It interacts within mitochondrial pathways involving oxidative phosphorylation and reactive oxygen species (ROS) regulation. It associates with other proteins such as NDUFS3 and NDUFS4 as part of the complex I formation process. Progressive assembly of complex I facilitated by NDUFAF4 is an important step in maintaining cellular energy metabolism.

Improper function or expression of NDUFAF4 leads to mitochondrial disorders including Leigh syndrome and other metabolic encephalopathies. Mutations in NDUFAF4 disrupt complex I assembly resulting in impaired mitochondrial respiration and energy production. This protein also relates to Parkinson’s disease where its involvement in complex I assembly connects it to proteins like Parkin which are associated with mitochondrial dysfunction in dopaminergic neurons.