Ndufab1-KO Mouse

Ndufab1, also known as mitochondrial acyl carrier protein, is a nuclear-encoded subunit of complex I of the mitochondrial respiratory chain. It plays a crucial role in mitochondrial bioenergetics, coordinating the assembly of respiratory complexes I, II, and III, as well as supercomplexes. It is involved in regulating iron-sulfur biosynthesis and complex I subunit stability, and is essential for mitochondrial energy and ROS metabolism [1,3,4].

Cardiac-specific deletion of Ndufab1 in mice led to defective bioenergetics, elevated ROS levels, progressive dilated cardiomyopathy, heart failure, and sudden death [1]. Skeletal muscle-specific ablation of Ndufab1 caused systemic disruption of glucose homeostasis, defective insulin signaling, growth arrest, and early death within 5 postnatal days [2].

In conclusion, Ndufab1 is a key regulator of mitochondrial metabolism. Gene-knockout mouse models have revealed its significance in preventing heart-related diseases like heart failure, as well as metabolic disorders such as obesity and insulin resistance. Understanding Ndufab1's functions provides potential therapeutic targets for these diseases.