[HTML][HTML] Identification and characterization of a common set of complex I assembly intermediates in mitochondria from patients with complex I deficiency
H Antonicka, I Ogilvie, T Taivassalo, RP Anitori… - Journal of Biological …, 2003 - ASBMB
Deficiencies in the activity of complex I (NADH: ubiquinone oxidoreductase) are an
important cause of human mitochondrial disease. Complex I is composed of at least 46
structural subunits that are encoded in both nuclear and mitochondrial DNA. Enzyme
deficiency can result from either impaired catalytic efficiency or an inability to assemble the
holoenzyme complex; however, the assembly process remains poorly understood. We have
used two-dimensional Blue-Native/SDS gel electrophoresis and a panel of 11 antibodies …
important cause of human mitochondrial disease. Complex I is composed of at least 46
structural subunits that are encoded in both nuclear and mitochondrial DNA. Enzyme
deficiency can result from either impaired catalytic efficiency or an inability to assemble the
holoenzyme complex; however, the assembly process remains poorly understood. We have
used two-dimensional Blue-Native/SDS gel electrophoresis and a panel of 11 antibodies …