Ubiquinol-Cytochrome C Reductase Core Protein II, also known as UQCRC2, is a crucial component of the mitochondrial respiratory chain. This protein is encoded by the UQCRC2 gene in humans and plays a significant role in cellular respiration and energy production.
UQCRC2 is a part of the ubiquinol-cytochrome c reductase complex, also known as Complex III or the cytochrome bc1 complex, which is a multisubunit transmembrane complex . This complex is integral to the mitochondrial electron transport chain, which drives oxidative phosphorylation . The primary function of UQCRC2 is to facilitate the transfer of electrons from ubiquinol to cytochrome c, a critical step in the production of ATP, the energy currency of the cell .
The mitochondrial respiratory chain consists of four multisubunit complexes (I-IV) that work together to transfer electrons derived from NADH and succinate to molecular oxygen . UQCRC2 is a core protein of Complex III, which is responsible for creating an electrochemical gradient across the inner mitochondrial membrane . This gradient drives the synthesis of ATP by ATP synthase .
Mutations or defects in the UQCRC2 gene can lead to mitochondrial complex III deficiency, nuclear type 5 . This deficiency can result in a range of clinical manifestations, including muscle weakness, neurological disorders, and metabolic abnormalities . Research has also shown that the expression of UQCRC2 is upregulated in multiple human tumors, suggesting a potential role in cancer progression .
Recombinant UQCRC2 is produced using recombinant DNA technology, which involves inserting the UQCRC2 gene into a suitable expression system, such as bacteria or yeast, to produce the protein in large quantities. This recombinant protein is used in various research applications to study its structure, function, and role in disease.