Rabbit Polyclonal UQCC2 Antibody

The UQCC2 antibody is a valuable tool for studying the Ubiquinol-cytochrome c reductase complex assembly factor 2 (UQCC2), a nuclear-encoded protein critical for the assembly and stability of mitochondrial respiratory chain Complex III (cytochrome bc1 complex). UQCC2 localizes to the mitochondrial matrix and interacts with UQCC1 to facilitate the early stages of Complex III biogenesis. Mutations in the UQCC2 gene are linked to mitochondrial disorders, including severe metabolic encephalopathies and growth deficiencies, often associated with impaired oxidative phosphorylation (OXPHOS) and elevated reactive oxygen species (ROS). Researchers utilize UQCC2 antibodies in techniques like Western blotting, immunofluorescence, and immunohistochemistry to investigate mitochondrial protein expression, subcellular localization, and molecular interactions in disease models. These studies are essential for understanding the pathogenesis of mitochondrial diseases, metabolic syndromes, and conditions linked to OXPHOS dysfunction. The antibody also aids in validating CRISPR/Cas9-edited cell lines or animal models with UQCC2 mutations, enabling mechanistic insights into energy metabolism defects and potential therapeutic strategies. Commercial UQCC2 antibodies are typically developed in rabbit or mouse hosts, with validation data confirming specificity for human, mouse, or rat homologs.