Cytochrome c oxidase-deficient patients have distinct subunit assembly profiles.
AuthorsHanson BJ, Carrozzo R, Piemonte F, Tessa A, Robinson BH, Capaldi RA
JournalJ Biol Chem
PubMed ID11278850
'Cytochrome c oxidase (COX) deficiency is the most common respiratory chain defect in childhood and is clinically heterogeneous. We report a study of six patients with COX deficiencies. Two of the patients had as yet undefined defects, three patients had Surf-1 mutations, and one patient had a 15-base pair deletion ... More
Simultaneous red/green dual fluorescence detection on electroblots using BODIPY TR-X succinimidyl ester and ELF 39 phosphate.
AuthorsMartin K, Hart C, Schulenberg B, Jones L, Patton WF
JournalProteomics
PubMed ID11987124
'A two-color fluorescence detection method is described based upon covalently coupling the succinimidyl ester of BODIPY TR-X dye to proteins immobilized on polyvinylidene difluoride membranes, followed by detection of target proteins using the fluorogenic, precipitating substrate ELF 39-phosphate in combination with alkaline phosphatase conjugated reporter molecules. This results in all ... More
Calcineurin regulates skeletal muscle metabolism via coordinated changes in gene expression.
AuthorsLong YC, Glund S, Garcia-Roves PM, Zierath JR
JournalJ Biol Chem
PubMed ID17107952
'The metabolic property of skeletal muscle adapts in response to an increased physiological demand by altering substrate utilization and gene expression. The calcium-regulated serine/threonine protein phosphatase calcineurin has been implicated in the transduction of motor neuron signals to alter gene expression programs in skeletal muscle. We utilized transgenic mice that ... More
Dysregulation of mitochondrial biogenesis in vascular endothelial and smooth muscle cells of aged rats.
AuthorsUngvari Z, Labinskyy N, Gupte S, Chander PN, Edwards JG, Csiszar A,
JournalAm J Physiol Heart Circ Physiol
PubMed ID18326800
Mitochondrial biogenesis is involved in the control of cell metabolism, signal transduction, and regulation of mitochondrial reactive oxygen species (ROS) production. Despite the central role of mitochondria in cellular aging and endothelial physiology, there are no studies extant investigating age-related alterations in mitochondrial biogenesis in blood vessels. Electronmicroscopy and confocal ... More
Resveratrol induces mitochondrial biogenesis in endothelial cells.
AuthorsCsiszar A, Labinskyy N, Pinto JT, Ballabh P, Zhang H, Losonczy G, Pearson K, de Cabo R, Pacher P, Zhang C, Ungvari Z,
JournalAm J Physiol Heart Circ Physiol
PubMed ID19429820
Pathways that regulate mitochondrial biogenesis are potential therapeutic targets for the amelioration of endothelial dysfunction and vascular disease. Resveratrol was shown to impact mitochondrial function in skeletal muscle and the liver, but its role in mitochondrial biogenesis in endothelial cells remains poorly defined. The present study determined whether resveratrol induces ... More
Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I.
AuthorsIuso A, Scacco S, Piccoli C, Bellomo F, Petruzzella V, Trentadue R, Minuto M, Ripoli M, Capitanio N, Zeviani M, Papa S
JournalJ Biol Chem
PubMed ID16478720
The pathogenic mechanism of a G44A nonsense mutation in the NDUFS4 gene and a C1564A mutation in the NDUFS1 gene of respiratory chain complex I was investigated in fibroblasts from human patients. As previously observed the NDUFS4 mutation prevented complete assembly of the complex and caused full suppression of the ... 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
Gaf-1, a gamma -SNAP-binding protein associated with the mitochondria.
AuthorsChen D, Xu W, He P, Medrano EE, Whiteheart SW
JournalJ Biol Chem
PubMed ID11278501
The role of alpha/beta-SNAP (Soluble NSF Attachment Protein) in vesicular trafficking is well established; however, the function of the ubiquitously expressed gamma-SNAP remains unclear. To further characterize the cellular role of this enigmatic protein, a two-hybrid screen was used to identify new, gamma-SNAP-binding proteins and to uncover potentially novel functions ... More
Simultaneous trichromatic fluorescence detection of proteins on Western blots using an amine-reactive dye in combination with alkaline phosphatase- and horseradish peroxidase-antibody conjugates.
AuthorsMartin K, Hart C, Liu J, Leung WY, Patton WF
JournalProteomics
PubMed ID12872222
Three-color fluorescence detection methods are described based upon covalently coupling the dye 2-methoxy-2,4-diphenyl-2(2H)-furanone (MDPF) to proteins immobilized on poly(vinylidene difluoride) (PVDF) membranes, followed by detection of target proteins using alkaline-phosphatase-conjugated reporter molecules in combination with the fluorogenic substrate 9H-(1,3-dichloro-9,9-dimethylacridin-2-one-7-yl) phosphate (DDAO-phosphate) as well as horseradish peroxidase-conjugated reporter molecules in combination ... More