IMMUNOCAPTURE AND FUNCTIONAL ASSAY FOR MITOCHONDRIAL COMPLEX I

摘要

Complex I defects are one of the most frequent causes of diseases and disorders associated with mitochondrial respiratory chain disorders, including genetically related diseases and late onset neurodegenerative diseases. The invention provides methods for using monoclonal antibodies that react with 39-, 30-, 20-, 18-, 15-, and 8-kDa subunits of Complex I to aid in diagnosis of Complex I deficiencies and that can be used for rapid screening to detect onset or stage of a variety of diseases. The invention further provides methods for screening agents, such as drugs, for their effect upon Complex I activity and for screening patients to detect those with impairment of Complex I activity and those suspected of having a late onset disease associated with post-translational modification of one or more subunits of Complex I.