Results 21 to 30 of about 66,093 (301)

The arabidopsis COX11 homolog is essential for cytochrome c oxidase activity [PDF]

, 2015
Members of the ubiquitous COX11 (cytochrome c oxidase 11) protein family are involved in copper delivery to the COX complex. In this work, we characterize the Arabidopsis thaliana COX11 homolog (encoded by locus At1g02410).
Mansilla, Natanael, Radin, Ivan, Rödel, Gerhard, Steinebrunner, Iris   +3 more
core   +2 more sources

Benign Cox Deficiency Myopathy

Pediatric Neurology Briefs, 2011
Clinical and pathological features of benign infantile mitochondrial cytochrome c oxidase (COX) deficiency were studied in 8 patients with the disease phenotype seen at the National Center of Neurology and Psychiatry, Tokyo, Japan.
J Gordon Millichap
doaj   +1 more source

COA6 facilitates cytochrome c oxidase biogenesis as thiol-reductase for copper metallochaperones in mitochondria.

, 2020
The mitochondrial cytochrome c oxidase, the terminal enzyme of the respiratory chain, contains heme and copper centers for electron transfer. The conserved COX2 subunit contains the CuA site, a binuclear copper center.
Aich, A., Bogeski, I., Chacinska, A., Chudenkova, M., Deckers, M., Dennerlein, S., Gibhardt, C., Oeljeklaus, S., Pacheu-Grau, D., Rehling, P., Warscheid, B., Wasilewski, M.   +11 more
core   +2 more sources

A mutant of Neurospora crassa deficient in cytochrome c heme lyase activity cannot import cytochrome c into mitochondria [PDF]

, 1988
The nuclear cyt-2-1 mutant of Neurospora crassa is characterized by a gross deficiency of cytochrome c (Bertrand, H., and Collins, R. A. (1978) Mol. Gen. Genet. 166, 1-13).
Drygas, Mariola E., Kwong, Patrick L., Nargang, Frank E., Neupert, Walter, Nicholson, Donald W.   +4 more
core   +1 more source

Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice

Nature Communications, 2016
Mutations in the translational activator of cytochrome c oxidase subunit I (TACO1) causes cytochrome c oxidase deficiency and Leigh Syndrome in patients.
Tara R. Richman, Henrik Spåhr, Judith A. Ermer, Stefan M. K. Davies, Helena M. Viola, Kristyn A. Bates, John Papadimitriou, Livia C. Hool, Jennifer Rodger, Nils-Göran Larsson, Oliver Rackham, Aleksandra Filipovska   +11 more
doaj   +1 more source

Progression of myopathology in Kearns-Sayre syndrome [PDF]

, 1992
We report on the progression of myopathology by comparing two biopsies from a patient with a Kearns-Sayre-Syndrome. The first biopsy was taken in 1979 and showed 10% ragged-red fibers.
B. Meurers, CT Moraes, H Reichmann, Heinz Reichmann, I Nonaka, JA Morgan-Hughes, M. Naumann, P. Seibel, R. Gold, T. Klopstock, U. Walter, V Dubowitz   +11 more
core   +1 more source

Intracytoplasmic Copper Homeostasis Controls Cytochrome c Oxidase Production

mBio, 2014
Copper is an essential micronutrient used as a metal cofactor by a variety of enzymes, including cytochrome c oxidase (Cox). In all organisms from bacteria to humans, cellular availability and insertion of copper into target proteins are tightly ...
Seda Ekici, Serdar Turkarslan, Grzegorz Pawlik, Andrew Dancis, Nitin S. Baliga, Hans-Georg Koch, Fevzi Daldal   +6 more
doaj   +1 more source

No excess of mitochondrial DNA deletions within muscle in progressive multiple sclerosis [PDF]

, 2013
BACKGROUND: Mitochondrial dysfunction is an established feature of multiple sclerosis (MS). We recently described high levels of mitochondrial DNA (mtDNA) deletions within respiratory enzyme-deficient (lacking mitochondrial respiratory chain complex IV ...
Abadi A, Amy K Reeve, Brierley EJ, Broadwater L, Campbell GR, Campbell GR, Campbell GR, Campbell GR, Don J Mahad, Doug M Turnbull, Dutta R, Dutta R, Graham R Campbell, Harding AE, Horvath R, Iryna Ziabreva, Johnson MA, Krishnan KJ, Kumleh HH, Larsson NG, Mahler A, McFarland R, Murphy JL, Nociti V, Palace J, Parise G, Patte M, Rahman S, Rahman S, Reeve AK, Richard Reynolds, Sakiyama Y, Taylor RW, Verny C, Witte ME, Witte ME, Yu-Wai-Man P   +36 more
core   +2 more sources

Potentially Diagnostic Electron Paramagnetic Resonance Spectra Elucidate the Underlying Mechanism of Mitochondrial Dysfunction in the Deoxyguanosine Kinase Deficient Rat Model of a Genetic Mitochondrial DNA Depletion Syndrome [PDF]

, 2016
A novel rat model for a well-characterized human mitochondrial disease, mitochondrial DNA depletion syndrome with associated deoxyguanosine kinase (DGUOK) deficiency, is described.
Bennett, Brian, Dimmock, David P., Friederich, Marisa W., Geurts, Aron M., Helbling, Daniel, Jarzembowski, Jason, Lawlor, Michael W., Meng, Hui, Van Hove, Johan L.K.   +8 more
core   +2 more sources

Mutations in SURF1 are important genetic causes of Leigh syndrome in Slovak patients

Endocrine Regulations, 2018
Objectives. Leigh syndrome is a progressive early onset neurodegenerative disease typically presenting with psychomotor regression, signs of brainstem and/or basal ganglia disease, lactic acidosis, and characteristic magnetic resonance imaging findings ...
Danis Daniel, Brennerova Katarina, Skopkova Martina, Kurdiova Timea, Ukropec Jozef, Stanik Juraj, Kolnikova Miriam, Gasperikova Daniela   +7 more
doaj   +1 more source