NDUFS1 Mouse mAb

NDUFS1 (NADH:Ubiquinone Oxidoreductase Core Subunit S1) is the largest core subunit of mitochondrial respiratory chain Complex I. Although encoded by the nuclear genome, it is post-translationally translocated to the inner mitochondrial membrane to exert its function. As a critical component of the catalytic core of Complex I, it contains multiple iron-sulfur clusters and directly participates in the key redox reaction that transfers electrons from NADH to ubiquinone, playing a vital role in maintaining mitochondrial energy metabolism. Dysfunction of this protein is closely associated with mitochondrial Complex I deficiency, and mutations in its encoding gene can lead to a diverse spectrum of mitochondrial diseases with variable clinical presentations, such as Leigh syndrome and progressive leukoencephalopathy, which can be life-threatening in severe cases.