Ndufab1-flox Mouse

NDUFAB1, also known as mitochondrial acyl carrier protein, is a crucial component in mitochondrial metabolism. It is a subunit of complex I in the mitochondrial respiratory chain and is involved in coordinating the assembly of respiratory complexes I, II, and III, as well as supercomplexes. This coordination occurs through regulating iron-sulfur biosynthesis and complex I subunit stability, thus playing a significant role in mitochondrial bioenergetics and related pathways [1,3,4].

In mouse models, cardiac-specific deletion of Ndufab1 led to defective bioenergetics, elevated ROS levels, progressive dilated cardiomyopathy, and eventually 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]. These gene knockout (KO) mouse models clearly demonstrate the essential role of Ndufab1 in maintaining normal physiological functions related to energy metabolism and metabolic regulation.

In conclusion, Ndufab1 is essential for mitochondrial energy and ROS metabolism by coordinating the assembly of respiratory complexes and supercomplexes. The KO mouse models have revealed its significance in preventing heart failure, obesity, and insulin resistance, highlighting its potential as a therapeutic target in these disease areas [1,2].