Abstract: Isolated antibody compositions for immunolabeling are described herein. These compositions overcome many of the limitations currently involved with immunolabeling such as low fluorescence and high noise signal.

Abstract: The present invention is based on the discovery that an antibody specific for PDH complex can be used to immunoprecitate PDH complex from the patient sample in an active state. Therefore the anti-PDH complex specific antibody can be used to determine the amount of and/or active state of PDH in a patient sample. The invention immunoassay methods for determining the amount and/or active state of PDH complex present in a patient sample are useful for screening to identify individuals having symptoms indicating malfunction of PDH complex. For example, the invention methods can be used to screen individuals for symptoms of onset of the diabetic state, such as insulitis, and for diagnosing late onset diseases, such as diabetes, Alzheimer's and the like.

Abstract: Provided herein is a library of monoclonal antibodies specific for native proteins and native protein complexes of the oxidative phosphorylation (OXPHOS) system (for example, Complex I, II, III, IV, or V, or any protein subunit of any of such complexes). Hybridomas expressing such antibodies and antibodies that competitively inhibit the binding of any such antibody (e.g., antibodies that bind the same or a sterically overlapping epitope) are also contemplated. Methods of using, and kits including, the disclosed antibodies are also provided. Antibodies, methods and kits described herein address a need in the art by providing immunological reagents and assays useful, at least, for detecting mitochondrial diseases associated with deficiencies or alterations in OXPHOS Complexes I, II, III, IV and/or V.