Antoni Barrientos, Andrea Zambrano, Alexander Tzagoloff,
... Search for more papers by this author Alexander Tzagoloff Alexander Tzagoloff Department of Biological Sciences, Columbia University, New ... Search for more papers by this author Alexander Tzagoloff Alexander Tzagoloff Department of Biological Sciences, Columbia University, New York, NY, USA Search for more papers by this author Author Information Antoni Barrientos 1,2, Andrea Zambrano2 and Alexander Tzagoloff1 1Department of Biological Sciences, Columbia University, New ...
Tópico(s): Plant biochemistry and biosynthesis
2004 - Springer Nature | The EMBO Journal
Alexander Tzagoloff, Anna Akai, Françoise Foury,
... in oligomycin-resistant mutants of Saccharomyces cerevisiae Alexander Tzagoloff, Alexander Tzagoloff The Public Health Research Institute, of the ... USASearch for more papers by this author Alexander Tzagoloff, Alexander Tzagoloff The Public Health Research Institute, of the City ...
Tópico(s): Peroxisome Proliferator-Activated Receptors
1976 - Wiley | FEBS Letters
Francisco G. Nóbrega, Alexander Tzagoloff,
... Brasil Search for more papers by this authorAlexander Tzagoloff, Alexander Tzagoloff Department of Biological Sciences, Columbia University, New ... Brasil Search for more papers by this authorAlexander Tzagoloff, Alexander Tzagoloff Department of Biological Sciences, Columbia University, New York, ...
Tópico(s): RNA and protein synthesis mechanisms
1980 - Wiley | FEBS Letters
Suzie W. Baba, Grigory I. Belogrudov, Justine C. Lee, Peter T. Lee, Jeff R. Strahan, Jennifer N. Shepherd, Catherine F. Clarke,
... yeast coq5 mutant strains were provided by Dr. Alexander Tzagoloff, Dept. of Biological Sciences, Columbia University. × W303-1ACH256- ... yeast coq5 mutant strains were provided by Dr. Alexander Tzagoloff, Dept. of Biological Sciences, Columbia University. × W303-1ACH256- ... yeast coq5 mutant strains were provided by Dr. Alexander Tzagoloff, Dept. of Biological Sciences, Columbia University. × W303-1ACH259- ... yeast coq5 mutant strains were provided by Dr. Alexander Tzagoloff, Dept. of Biological Sciences, Columbia University. × W303-1ACH259- ...
Tópico(s): Biochemical Acid Research Studies
2004 - Elsevier BV | Journal of Biological Chemistry
Malgorzata Rak, Samanta Gokova, Alexander Tzagoloff,
... Search for more papers by this author Alexander Tzagoloff Corresponding Author Alexander Tzagoloff Department of Biological Sciences, Columbia University, New ... Search for more papers by this author Alexander Tzagoloff Corresponding Author Alexander Tzagoloff Department of Biological Sciences, Columbia University, New York, ... author Author Information Malgorzata Rak1, Samanta Gokova1 and Alexander Tzagoloff 1 1Department of Biological Sciences, Columbia University, New ...
Tópico(s): Photosynthetic Processes and Mechanisms
2011 - Springer Nature | The EMBO Journal
... Search for more papers by this author Alexander Tzagoloff Corresponding Author Alexander Tzagoloff Department of Biological Sciences, Columbia University, New ... Search for more papers by this author Alexander Tzagoloff Corresponding Author Alexander Tzagoloff Department of Biological Sciences, Columbia University, New York, ... author Author Information Antoni Barrientos1, Daniel Korr1 and Alexander Tzagoloff 1 1Department of Biological Sciences, Columbia University, New ...
Tópico(s): ATP Synthase and ATPases Research
2002 - Springer Nature | The EMBO Journal
... 521-526 ASSEMBLY OF INNER MEMBRANE COMPLEXES* Alexander Tzagoloff, Alexander Tzagoloff Department of Biochemistry The Public Health Research ... 10016Search for more papers by this author Alexander Tzagoloff, Alexander Tzagoloff Department of Biochemistry The Public Health Research Institute ...
Tópico(s): Coenzyme Q10 studies and effects
1974 - Wiley | Annals of the New York Academy of Sciences
Malgorzata Rak, Chen Su, Jonathan Xu, Ricardo Azpiroz, Angela Mohan Singh, Alexander Tzagoloff,
Expression of the mitochondrially encoded ATP6 and ATP8 genes is translationally regulated by F1 ATPase. We report a translational repressor (Smt1p) of the ATP6/8 mRNA that, when mutated, restores translation of the encoded Atp6p and Atp8p subunits of the ATP synthase. Heterozygous smt1 mutants fail to rescue the translation defect, indicating that the mutations are recessive. Smt1p is an intrinsic inner membrane protein, which, based on its sedimentation, has a native size twice that of the monomer. ...
Tópico(s): RNA and protein synthesis mechanisms
2016 - American Society for Cell Biology | Molecular Biology of the Cell
Malgorzata Rak, Paule Bénit, Dominique Chrétien, Juliette Bouchereau, Manuel Schiff, Riyad El‐Khoury, Alexander Tzagoloff, Pierre Rustin,
As with other mitochondrial respiratory chain components, marked clinical and genetic heterogeneity is observed in patients with a cytochrome c oxidase deficiency. This constitutes a considerable diagnostic challenge and raises a number of puzzling questions. So far, pathological mutations have been reported in more than 30 genes, in both mitochondrial and nuclear DNA, affecting either structural subunits of the enzyme or proteins involved in its biogenesis. In this review, we discuss the possible ...
Tópico(s): Metabolism and Genetic Disorders
2016 - Portland Press | Clinical Science
Manuel Schiff, Alice Veauville‐Merllié, Chen Su, Alexander Tzagoloff, Malgorzata Rak, Hélène Ogier de Baulny, Audrey Boutron, Hélène Smedts-Walters, Norma B. Romero, Odile Rigal, Pierre Rustin, Christine Vianey‐Saban, Cécile Acquaviva,
A patient with late-onset exercise intolerance had haploinsufficiency of SLC25A32, which encodes the human mitochondrial flavin adenine dinucleotide transporter. The patient's symptoms were highly responsive to oral supplementation with riboflavin.
Tópico(s): Amino Acid Enzymes and Metabolism
2016 - Massachusetts Medical Society | New England Journal of Medicine
Chen-Hsien Su, Gavin P. McStay, Alexander Tzagoloff,
Yeast cytochrome oxidase (COX) was previously inferred to assemble from three modules, each containing one of the three mitochondrially encoded subunits and a different subset of the eight nuclear gene products that make up this respiratory complex. Pull-down assays of pulse-labeled mitochondria enabled us to characterize Cox3p subassemblies that behave as COX precursors and contain Cox4p, Cox7p, and Cox13p. Surprisingly, Cox4p is a constituent of two other complexes, one of which was previously ...
Tópico(s): Plant biochemistry and biosynthesis
2014 - American Society for Cell Biology | Molecular Biology of the Cell
Gavin P. McStay, Chen Su, Alexander Tzagoloff,
Cox14p and Coa3p have been shown to regulate translation of the mitochondrial COX1 mRNA and to be required for assembly of cytochrome oxidase. We present evidence that Cox14p and Coa3p stabilize previously identified Cox1p intermediates and that in the absence of either protein, Cox1p aggregates with itself and other mitochondrial gene products, including cytochrome b , Var1p and Cox2p. Our evidence suggests that Cox1p assembly intermediates are in close proximity to other mitochondrially translated ...
Tópico(s): Photosynthetic Processes and Mechanisms
2013 - Wiley | FEBS Letters
Gavin P. McStay, Chen-Hsien Su, Susan M. Thomas, Jonathan Xu, Alexander Tzagoloff,
Mitochondrial-encoded Cox1p, one of the three core subunits of yeast cytochrome oxidase (COX), was previously shown to associate with regulatory proteins and nuclear-encoded subunits into five high molecular weight complexes that were proposed to constitute the pathway for biogenesis of the Cox1p assembly module. One of the intermediates (D5) was inferred, but not directly shown to exist. In the present study mitochondria of strains expressing C-terminal-tagged subunits of COX that had not been looked ...
Tópico(s): Fungal and yeast genetics research
2013 - Elsevier BV | Journal of Biological Chemistry
Pauline Gaignard, Minal Menezes, Manuel Schiff, Aurélien Bayot, Malgorzata Rak, Hélène Ogier de Baulny, Chen-Hsien Su, Martine Gilleron, Anne Lombès, Heni Abida, Alexander Tzagoloff, Lisa G. Riley, Sandra T. Cooper, Kym Mina, Padma Sivadorai, Mark R. Davis, Richard J.N. Allcock, Nina Kresoje, Nigel G. Laing, David R. Thorburn, Abdelhamid Slama, John Christodoulou, Pierre Rustin,
Many individuals with abnormalities of mitochondrial respiratory chain complex III remain genetically undefined. Here, we report mutations (c.288G>T [p.Trp96Cys] and c.643C>T [p.Leu215Phe]) in CYC1, encoding the cytochrome c1 subunit of complex III, in two unrelated children presenting with recurrent episodes of ketoacidosis and insulin-responsive hyperglycemia. Cytochrome c1, the heme-containing component of complex III, mediates the transfer of electrons from the Rieske iron-sulfur protein to cytochrome ...
Tópico(s): Adipose Tissue and Metabolism
2013 - Elsevier BV | The American Journal of Human Genetics
Produção Nacional
Revisado por pares
Cleverson Busso, Erich Birelli Tahara, Renata Ogusucu, Ohára Augusto, José Ribamar Ferreira‐Junior, Alexander Tzagoloff, Alicia J. Kowaltowski, Mário H. Barros,
Deletion of COQ10 in Saccharomyces cerevisiae elicits a respiratory defect characterized by the absence of cytochrome c reduction, which is correctable by the addition of exogenous diffusible coenzyme Q(2). Unlike other coq mutants with hampered coenzyme Q(6) (Q(6) ) synthesis, coq10 mutants have near wild-type concentrations of Q(6). In the present study, we used Q-cycle inhibitors of the coenzyme QH(2)-cytochrome c reductase complex to assess the electron transfer properties of coq10 cells. Our results ...
Tópico(s): Free Radicals and Antioxidants
2010 - Wiley | FEBS Journal
Produção Nacional
Revisado por pares
Mariana A. Zampol, Cleverson Busso, Fernando Gomes, José Ribamar Ferreira‐Junior, Alexander Tzagoloff, Mário H. Barros,
COQ10 deletion in Saccharomyces cerevisiae elicits a defect in mitochondrial respiration correctable by addition of coenzyme Q2. Rescue of respiration by Q2 is a characteristic of mutants blocked in coenzyme Q6 synthesis. Unlike Q6 deficient mutants, mitochondria of the coq10 null mutant have wild-type concentrations of Q6. The physiological significance of earlier observations that purified Coq10p contains bound Q6 was examined in the present study by testing the in vivo effect of over-expression ...
Tópico(s): Mitochondrial Function and Pathology
2010 - Elsevier BV | Biochemical and Biophysical Research Communications
Malgorzata Rak, Alexander Tzagoloff,
The ATP synthase of yeast mitochondria is composed of 17 different subunit polypeptides. We have screened a panel of ATP synthase mutants for impaired expression of Atp6p, Atp8p, and Atp9p, the only mitochondrially encoded subunits of ATP synthase. Our results show that translation of Atp6p and Atp8p is activated by F(1) ATPase (or assembly intermediates thereof). Mutants lacking the alpha or beta subunits of F(1), or the Atp11p and Atp12p chaperones that promote F(1) assembly, have normal levels ...
Tópico(s): RNA and protein synthesis mechanisms
2009 - National Academy of Sciences | Proceedings of the National Academy of Sciences
Xiaomei Zeng, Mário H. Barros, T. S. Shul'man, Alexander Tzagoloff,
We report a new nuclear gene, designated ATP25 (reading frame YMR098C on chromosome XIII), required for expression of Atp9p (subunit 9) of the Saccharomyces cerevisiae mitochondrial proton translocating ATPase. Mutations in ATP25 elicit a deficit of ATP9 mRNA and of its translation product, thereby preventing assembly of functional F(0). Unlike Atp9p, the other mitochondrial gene products, including ATPase subunits Atp6p and Atp8p, are synthesized normally in atp25 mutants. Northern analysis of mitochondrial ...
Tópico(s): RNA modifications and cancer
2008 - American Society for Cell Biology | Molecular Biology of the Cell
Malgorzata Rak, Xiaomei Zeng, Jean-Jacques Brière, Alexander Tzagoloff,
Respiratory deficient mutants of Saccharomyces cerevisiae have been instrumental in identifying an increasing number of nuclear gene products that promote pre- and post-translational steps of the pathway responsible for biogenesis of the mitochondrial ATP synthase. In this article we have attempted to marshal current information about the functions of such accessory factors and the roles they play in expression and assembly of the mitochondrially encoded subunits of the ATP synthase. We also discuss ...
Tópico(s): RNA modifications and cancer
2008 - Elsevier BV | Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
Kevin M. Rigby, Paul A. Cobine, Oleh Khalimonchuk, Dennis R. Winge,
... 1, cox17::TRP1) was generously provided by Dr. Alexander Tzagoloff. Growth Media—Yeast strains cultured in liquid medium ...
Tópico(s): Photosynthetic Processes and Mechanisms
2008 - Elsevier BV | Journal of Biological Chemistry
Flavia Fontanesi, Can Jin, Alexander Tzagoloff, Antoni Barrientos,
Cell survival and energy production requires a functional mitochondrial respiratory chain. Biogenesis of cytochrome c oxidase (COX), the last enzyme of the mitochondrial respiratory chain, is a very complicated process and requires the assistance of a large number of accessory factors. Defects in COX assembly alter cellular respiration and produce severe human encephalomyopathies. Mutations in SURF1, a COX assembly factor of exact unknown function, produce Leigh's syndrome (LS), the most frequent ...
Tópico(s): Photosynthetic Processes and Mechanisms
2007 - Oxford University Press | Human Molecular Genetics
Xiaomei Zeng, Róża Kucharczyk, Jean-Paul di Rago, Alexander Tzagoloff,
Atp6p (subunit 6) of the Saccharomyces cerevisiae mitochondrial ATPase is synthesized with an N-terminal 10-amino acid presequence that is cleaved during assembly of the complex. This study has examined the role of the Atp6p presequence in the function and assembly of the ATPase complex. Two mutants were constructed in which the codons for amino acids 2-9 or 2-10 of the Atp6p precursor were deleted from the mitochondrial ATP6 gene. The concentration of Atp6p and ATPase complex was approximately 2 ...
Tópico(s): Photosynthetic Processes and Mechanisms
2007 - Elsevier BV | Journal of Biological Chemistry
Can Jin, Antoni Barrientos, Charles B. Epstein, Ronald A. Butow, Alexander Tzagoloff,
E153 is a respiratory deficient mutant of Saccharomyces cerevisiae with a mutation in the active site of the Sit4p protein phosphatase. Measurements of mitochondrial respiration and cytochromes indicate that the mutation suppresses glucose repression. The escape from catabolite repression is accompanied by a marked reduction of the transcriptional repressor Mig1p. The presence of normal levels of MIG1 mRNA in the mutant and its association with the polysome fraction suggests that depletion of Mig1p ...
Tópico(s): Ubiquitin and proteasome pathways
2007 - Wiley | FEBS Letters
Xiaomei Zeng, Audrey Hourset, Alexander Tzagoloff,
Abstract Mutations in the Saccharomyces cerevisiae ATP22 gene were previously shown to block assembly of the F0 component of the mitochondrial proton-translocating ATPase. Further inquiries into the function of Atp22p have revealed that it is essential for translation of subunit 6 of the mitochondrial ATPase. The mutant phenotype can be partially rescued by the presence in the same cell of wild-type mitochondrial DNA and a ρ− deletion genome in which the 5′-UTR, first exon, and first intron of COX1 ...
Tópico(s): RNA and protein synthesis mechanisms
2006 - Oxford University Press | Genetics
Andrea Zambrano, Flavia Fontanesi, A. Solans, Rodrigo Leite de Oliveira, Thomas D. Fox, Alexander Tzagoloff, Antoni Barrientos,
Expression of yeast mitochondrial genes depends on specific translational activators acting on the 5'-untranslated region of their target mRNAs. Mss51p is a translational factor for cytochrome c oxidase subunit 1 (COX1) mRNA and a key player in down-regulating Cox1p expression when subunits with which it normally interacts are not available. Mss51p probably acts on the 5'-untranslated region of COX1 mRNA to initiate translation and on the coding sequence itself to facilitate elongation. Mss51p binds ...
Tópico(s): Photosynthetic Processes and Mechanisms
2006 - American Society for Cell Biology | Molecular Biology of the Cell
Xiaomei Zeng, Walter Neupert, Alexander Tzagoloff,
In the present study we have identified a new metalloprotease encoded by the nuclear ATP23 gene of Saccharomyces cerevisiae that is essential for expression of mitochondrial ATPase (F(1)-F(O) complex). Mutations in ATP23 cause the accumulation of the precursor form of subunit 6 and prevent assembly of F(O). Atp23p is associated with the mitochondrial inner membrane and is conserved from yeast to humans. A mutant harboring proteolytically inactive Atp23p accumulates the subunit 6 precursor but is ...
Tópico(s): Enzyme Structure and Function
2006 - American Society for Cell Biology | Molecular Biology of the Cell
Produção Nacional
Revisado por pares
Mário H. Barros, Alisha Johnson, Peter Gin, Beth N. Marbois, Catherine F. Clarke, Alexander Tzagoloff,
Deletion of the Saccharomyces cerevisiae gene YOL008W, here referred to as COQ10, elicits a respiratory defect as a result of the inability of the mutant to oxidize NADH and succinate. Both activities are restored by exogenous coenzyme Q2. Respiration is also partially rescued by COQ2, COQ7, or COQ8/ABC1, when these genes are present in high copy. Unlike other coq mutants, all of which lack Q6, the coq10 mutant has near normal amounts of Q6 in mitochondria. Coq10p is widely distributed in bacteria ...
Tópico(s): Biochemical Acid Research Studies
2005 - Elsevier BV | Journal of Biological Chemistry
Mário H. Barros, Alan M. Myers, Sarah Van Driesche, Alexander Tzagoloff,
In most strains of Saccharomyces cerevisiae the mitochondrial gene COX1, for subunit 1 of cytochrome oxidase, contains multiple exons and introns. Processing of COX1 primary transcript requires accessory proteins factors, some of which are encoded by nuclear genes and others by reading frames residing in some of the introns of the COX1 and COB genes. Here we show that the low molecular weight protein product of open reading frame YLR204W, for which we propose the name COX24, is also involved in ...
Tópico(s): Ubiquitin and proteasome pathways
2005 - Elsevier BV | Journal of Biological Chemistry
Sharon H. Ackerman, Alexander Tzagoloff,
This chapter briefly reviews some recent advances in the areas of the subunit structure and the different functions of the ATP synthase, but focuses mainly on the roles of mitochondrial and nuclear gene products in biogenesis of the mitochondrial enzyme. Most of the discussion related to biogenesis will be centered on the ATP synthase of Saccharomyces cerevisiae as it is studies of this particular enzyme that have provided much of the currently available information on this topic. As a facultative ...
Tópico(s): Photosynthetic Processes and Mechanisms
2005 - Academic Press | Progress in nucleic acid research and molecular biology
Alisha Johnson, Peter Gin, Beth N. Marbois, Edward J. Hsieh, Mian Wu, Mário H. Barros, Catherine F. Clarke, Alexander Tzagoloff,
Currently, eight genes are known to be involved in coenzyme Q6 biosynthesis in Saccharomyces cerevisiae. Here, we report a new gene designated COQ9 that is also required for the biosynthesis of this lipoid quinone. The respiratory-deficient pet mutant C92 was found to be deficient in coenzyme Q and to have low mitochondrial NADH-cytochrome c reductase activity, which could be restored by addition of coenzyme Q2. The mutant was used to clone COQ9, corresponding to reading frame YLR201c on chromosome ...
Tópico(s): Plant biochemistry and biosynthesis
2005 - Elsevier BV | Journal of Biological Chemistry