Alexa Fluor® 488 Anti-Ndufs4 antibody [EP7832]

Supplementary info

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

Activity summary

Ndufs4 also known as NADH:Ubiquinone Oxidoreductase Subunit S4 is an important component of mitochondrial complex I which is part of the electron transport chain. It has a molecular mass of approximately 18 kDa. The protein is mainly found in the inner mitochondrial membrane where it plays an essential role in cellular respiration by transferring electrons from NADH to ubiquinone. Ndufs4 is widely expressed in tissues with high energy demands such as the brain and heart.

Biological function summary

Ndufs4 functions as a part of mitochondrial complex I a large protein enzyme complex critical for oxidative phosphorylation. The complex comprises multiple subunits of which Ndufs4 is a small but essential part. By participating in electron transfer and proton pumping Ndufs4 supports ATP synthesis which is the main energy currency of the cell. Deficiency in Ndufs4 leads to dysfunctional energy production linked with metabolic disorders.

Pathways

The protein is an integral part of the respiratory chain pathway where it supports oxidative phosphorylation enabling effective ATP production. Within these pathways Ndufs4 interacts with other complex I subunits including Ndufs1 and Ndufa9 which stabilize electron transport. Disruptions in this chain can affect cellular energy metabolism and have downstream effects on various cellular processes.

Associated diseases and disorders

Mutations in the Ndufs4 gene are associated with Leigh syndrome and mitochondrial complex I deficiency. Leigh syndrome is a severe neurological disorder characterized by progressive loss of mental and movement abilities. Ndufs4 mutations also lead to complex I deficiency causing diverse symptoms ranging from neurological impairments to muscle weakness. These disorders highlight the critical link between Ndufs4 and proper mitochondrial function. The protein's connection to other complex I subunits such as Ndufs1 emphasizes its importance in maintaining regular cellular functions and preventing mitochondrial diseases.