Recombinant Human NDUFB4 protein (denatured) (His tag N-Terminus)

Supplementary information

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

NDUFB4 also known as NADH: ubiquinone oxidoreductase subunit B4 plays a role in the mitochondrial respiratory chain complex I. Weighing approximately 15 kDa NDUFB4 is expressed in mitochondria across various cell types. It couples electron transfer from NADH to ubiquinone thereby contributing to energy production process in cells. NDUFB4 is important in maintaining mitochondrial functionality due to its role in the electron transport chain.

Biological function summary

NADH: ubiquinone oxidoreductase subunit B4 is part of the NADH dehydrogenase complex I. This complex is involved in the initial step of the mitochondrial electron transport chain. NDUFB4 supports the bioenergetic functions of cells by facilitating redox reactions. It also helps establish the electrochemical gradient needed for ATP synthesis. This protein interacts synergistically with other subunits in complex I to ensure efficient energy conversion.

Pathways

NADH dehydrogenase complex I which includes NDUFB4 is central in oxidative phosphorylation. This process supports energy production by converting NADH and FADH2 into ATP. NDUFB4 is integrally linked with proteins like NDUFS2 and NDUFV1 within this pathway ensuring electron transport is seamless. Complex I contributes to metabolic processes like the TCA cycle by feeding the mitochondria with necessary energy intermediates.

Malfunction of NDUFB4 in complex I links to mitochondrial diseases such as Leigh syndrome. This syndrome affects central nervous system development. Mitochondrial encephalopathies are another group where NDUFB4 involvement is observed. In these conditions proteins like NDUFA1 and mitochondrial DNA mutations can manifest similar neurodegenerative symptoms due to impaired oxidative phosphorylation. Therefore understanding NDUFB4’s function helps in identifying therapeutic interventions for these disorders.