New antibodies specific for oxidative phosphorylation offer an insight into the causes of mitochondrial disease
Cambridge BioScience has launched a new range of subunit-specific mouse monoclonal antibodies for the study of oxidative phosphorylation (OxPhos).
These antibodies provide a valuable tool for the investigation of mitochondrial biogenesis and OxPhos-related diseases.
Once thought to be very rare, it is now clear that OxPhos deficiencies are an important cause of a wide range of neurological, neuromuscular, cardiac and endocrine disorders, and also some cancers.
The OxPhos antibodies, developed by Molecular Probes, bind monospecifically to the corresponding protein complex of the human oxidative phosphorylation system.
They can be used in a variety of assay formats including solid-phase binding of native proteins, Western blots of denatured proteins and immunohistochemistry of fixed tissues.
OxPhos monoclonal antibodies will prove valuable for both experimental cell biology and for research into human disease states.
OxPhos in mammalian mitochondria is catalysed by five membrane-bound protein complexes.
The complexes are composed of multiple subunits, some of which are encoded in the mitochondria while others are encoded in the nucleus.
Assembly of each complex involves a coordinated association of prosthetic groups with some polypeptides made in the mitochondrion and others made in the cytosol and then translocated to the organelle.
This process is complex and poorly understood but it is known that various assembly factors are required, each of which is specific for a particular complex.
Defects in assembly of one or more of these complexes contribute to several described mitochondrial diseases and possibly Alzheimer's and Parkinson's disease.
