Anti-NDUFS8 antibody [EPR11892]

Supplementary info

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

Activity summary

The NDUFS8 protein also known as NADH:ubiquinone oxidoreductase core subunit S8 serves an important function in the mitochondrial electron transport chain. It weighs approximately 23 kDa and is expressed in many tissues due to its essential role in energy production. NDUFS8 is a part of the larger mitochondrial complex I where it plays a role in transferring electrons derived from NADH to ubiquinone. Through this process NDUFS8 contributes to the creation of a proton gradient used for ATP synthesis.

Biological function summary

The NDUFS8 protein functions as an integral component of the mitochondrial respiratory complex I. Complex I is essential for cellular respiration and contributes to the production of ATP which cells use as an energy source. NDUFS8 helps facilitate the electron transport chain as a subunit of this multi-protein complex ensuring effective electron transfer and energy conversion within the mitochondria.

Pathways

The NDUFS8 protein operates within the oxidative phosphorylation pathway a major energy-producing pathway in cells. It also has connections with the tricarboxylic acid (TCA) cycle where NADH is generated and enters the electron transport chain through NDUFS8. The NDUFS8 protein collaborates with other subunits of complex I such as NDUFV1 and NDUFV2 to ensure proper electron flow and energy production.

Associated diseases and disorders

Mutations in the NDUFS8 protein can lead to mitochondrial disorders such as Leigh syndrome and mitochondrial encephalomyopathy. These conditions are characterized by severe energy production deficiencies due to impaired function of complex I. NDUFS8 mutations can interact with other proteins implicated in these disorders including NDUFAF2 which is involved in the assembly of complex I further disrupting normal mitochondrial activity and energy metabolism.