The topology of VDAC as probed by biotin modification.
AuthorsSong J, Midson C, Blachly-Dyson E, Forte M, Colombini M
JournalJ Biol Chem
PubMed ID9733730
'The outer membrane of mitochondria contains channels called VDAC (mitochondrial porin), which are formed by a single 30-kDa protein. Cysteine residues introduced by site-directed mutagenesis at sites throughout Neurospora crassa VDAC (naturally devoid of cysteine) were specifically biotinylated prior to reconstitution into planar phospholipid membranes. From previous studies, binding of ... More
Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns.
AuthorsTriepels RH, Hanson BJ, van den Heuvel LP, Sundell L, Marusich MF, Smeitink JA, Capaldi RA
JournalJ Biol Chem
PubMed ID11112787
Complex I defects are one of the most frequent causes of mitochondrial respiratory chain disorders. Therefore, it is important to find new approaches for detecting and characterizing Complex I deficiencies. In this paper, we introduce a new set of monoclonal antibodies that react with 39-, 30-, 20-, 18-, 15-, and ... More
An immunocytochemical approach to detection of mitochondrial disorders.
Mitochondrial disorders can lead to a confusing array of symptoms, which frequently makes a diagnosis difficult. Traditional approaches to such diagnoses are based on enzyme activity assays, with further characterization provided by genetic analysis. However, these methods require relatively large sample sizes, are time-consuming, labor-intensive, and show variability between laboratories. ... More