Temat: "Iron binding proteins" - OpacWWW

Skocz do pozycji: 1.

Tytuł:: Quantification of human mature frataxin protein expression in nonhuman primate hearts after gene therapy.
Autorzy:: Rojsajjakul T; Penn/CHOP Friedreich's Ataxia Center of Excellence and Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Hordeaux JJ; Gene Therapy Program, Departments of Medicine and Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Choudhury GR; Gene Therapy Program, Departments of Medicine and Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Hinderer CJ; Gene Therapy Program, Departments of Medicine and Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Mesaros C; Penn/CHOP Friedreich's Ataxia Center of Excellence and Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Wilson JM; Gene Therapy Program, Departments of Medicine and Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA. .
Blair IA; Penn/CHOP Friedreich's Ataxia Center of Excellence and Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA. .; Pokaż więcej
Źródło:: Communications biology [Commun Biol] 2023 Oct 27; Vol. 6 (1), pp. 1093. Date of Electronic Publication: 2023 Oct 27.
Typ publikacji:: Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:: Iron-Binding Proteins*/genetics
Friedreich Ataxia*/genetics
Friedreich Ataxia*/therapy
Friedreich Ataxia*/metabolism
Animals ; Humans ; Macaca mulatta ; Heart ; Genetic Therapy ; Frataxin

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Skocz do pozycji: 2.

Tytuł:: Expression and processing of mature human frataxin after gene therapy in mice.
Autorzy:: Rojsajjakul T; Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, Penn/CHOP Friedreich's Ataxia Center of Excellence, Center of Excellence in Environmental Toxicology, University of Pennsylvania, Philadelphia, PA, USA.
Selvan N; LEXEO Therapeutics, Inc, New York, NY, USA.
De B; Department of Genetic Medicine, Weill Cornell College of Medicine, New York, NY, USA.
Rosenberg JB; Department of Genetic Medicine, Weill Cornell College of Medicine, New York, NY, USA.
Kaminsky SM; Department of Genetic Medicine, Weill Cornell College of Medicine, New York, NY, USA.
Sondhi D; Department of Genetic Medicine, Weill Cornell College of Medicine, New York, NY, USA.
Janki P; LEXEO Therapeutics, Inc, New York, NY, USA.
Crystal RG; Department of Genetic Medicine, Weill Cornell College of Medicine, New York, NY, USA.
Mesaros C; Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, Penn/CHOP Friedreich's Ataxia Center of Excellence, Center of Excellence in Environmental Toxicology, University of Pennsylvania, Philadelphia, PA, USA.
Khanna R; LEXEO Therapeutics, Inc, New York, NY, USA.
Blair IA; Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, Penn/CHOP Friedreich's Ataxia Center of Excellence, Center of Excellence in Environmental Toxicology, University of Pennsylvania, Philadelphia, PA, USA. .; Pokaż więcej
Źródło:: Scientific reports [Sci Rep] 2024 Apr 10; Vol. 14 (1), pp. 8391. Date of Electronic Publication: 2024 Apr 10.
Typ publikacji:: Journal Article
MeSH Terms:: Frataxin*
Friedreich Ataxia*/therapy
Friedreich Ataxia*/drug therapy
Humans ; Animals ; Mice ; Heart ; Protein Processing, Post-Translational ; Liver/metabolism ; Genetic Therapy ; Iron-Binding Proteins/metabolism

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Skocz do pozycji: 3.

Tytuł:: Searching for Frataxin Function: Exploring the Analogy with Nqo15, the Frataxin-like Protein of Respiratory Complex I from Thermus thermophilus .
Autorzy:: Doni D; Department of Biology, University of Padova, 35121 Padova, Italy.
Cavallari E; Department of Biology, University of Padova, 35121 Padova, Italy.; Grenoble Alpes University, CNRS, CEA, INRAE, IRIG-LPCV, 38000 Grenoble, France.
Noguera ME; Department of Physiology and Molecular and Cellular Biology, Institute of Biosciences, Biotechnology and Translational Biology (iB3), Faculty of Exact and Natural Sciences, University of Buenos Aires, Intendente Güiraldes 2160, Buenos Aires C1428EG, Argentina.; Institute of Biological Chemistry and Physical Chemistry, Dr Alejandro Paladini (UBA-CONICET), University of Buenos Aires, Junín 956, Buenos Aires 1113AAD, Argentina.; Department of Science and Technology, National University of Quilmes, Roque Saenz Peña 352, Bernal B1876BXD, Argentina.
Gentili HG; Department of Physiology and Molecular and Cellular Biology, Institute of Biosciences, Biotechnology and Translational Biology (iB3), Faculty of Exact and Natural Sciences, University of Buenos Aires, Intendente Güiraldes 2160, Buenos Aires C1428EG, Argentina.
Cavion F; Department of Biology, University of Padova, 35121 Padova, Italy.
Parisi G; Department of Science and Technology, National University of Quilmes, Roque Saenz Peña 352, Bernal B1876BXD, Argentina.
Fornasari MS; Department of Science and Technology, National University of Quilmes, Roque Saenz Peña 352, Bernal B1876BXD, Argentina.
Sartori G; Department of Biomedical Sciences, University of Padova, 35121 Padova, Italy.
Santos J; Department of Physiology and Molecular and Cellular Biology, Institute of Biosciences, Biotechnology and Translational Biology (iB3), Faculty of Exact and Natural Sciences, University of Buenos Aires, Intendente Güiraldes 2160, Buenos Aires C1428EG, Argentina.
Bellanda M; Department of Chemical Sciences, University of Padova, 35131 Padova, Italy.; Consiglio Nazionale delle Ricerche Institute of Biomolecular Chemistry, 35131 Padova, Italy.
Carbonera D; Department of Chemical Sciences, University of Padova, 35131 Padova, Italy.
Costantini P; Department of Biology, University of Padova, 35121 Padova, Italy.
Bortolus M; Department of Chemical Sciences, University of Padova, 35131 Padova, Italy.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2024 Feb 05; Vol. 25 (3). Date of Electronic Publication: 2024 Feb 05.
Typ publikacji:: Journal Article
MeSH Terms:: Frataxin*
Friedreich Ataxia*/metabolism
Humans ; Electron Transport Complex I/metabolism ; Thermus thermophilus/metabolism ; Molecular Dynamics Simulation ; Iron/metabolism ; Iron-Binding Proteins/metabolism

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Skocz do pozycji: 4.

Tytuł:: Evolution of an Iron-Detoxifying Protein: Eukaryotic and Rickettsia Frataxins Contain a Conserved Site Which Is Not Present in Their Bacterial Homologues.
Autorzy:: Alves R; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.
Pazos-Gil M; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.
Medina-Carbonero M; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.
Sanz-Alcázar A; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.
Delaspre F; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.
Tamarit J; Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, IRBLleida, Universitat de Lleida, 25001 Lleida, Spain.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2022 Oct 29; Vol. 23 (21). Date of Electronic Publication: 2022 Oct 29.
Typ publikacji:: Journal Article
MeSH Terms:: Iron-Binding Proteins*/chemistry
Iron-Binding Proteins*/metabolism
Neurodegenerative Diseases*
Rickettsia*/metabolism
Humans ; Bacterial Proteins/chemistry ; Bacterial Proteins/metabolism ; Escherichia coli/metabolism ; Escherichia coli Proteins/genetics ; Eukaryota/metabolism ; Friedreich Ataxia/genetics ; Friedreich Ataxia/metabolism ; Iron/metabolism ; Tyrosine/metabolism ; Mitochondria/metabolism ; Mitochondria/microbiology ; Frataxin

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Skocz do pozycji: 5.

Tytuł:: Modelling Protein Plasticity: The Example of Frataxin and Its Variants.
Autorzy:: Botticelli S; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.
La Penna G; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.; Istituto di Chimica dei Composti Organometallici, Consiglio Nazionale delle Ricerche, Via Madonna del Piano 10, I-50019 Firenze, Italy.
Nobili G; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.
Rossi G; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.; Centro Fermi-Museo Storico della Fisica e Centro Studi e Ricerche E. Fermi, I-00184 Roma, Italy.
Stellato F; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.; Centro Fermi-Museo Storico della Fisica e Centro Studi e Ricerche E. Fermi, I-00184 Roma, Italy.
Morante S; Dipartimento di Fisica, Università di Roma Tor Vergata and Sezione di Roma Tor Vergata, INFN, Via della Ricerca Scientifica 1, I-00133 Roma, Italy.; Pokaż więcej
Źródło:: Molecules (Basel, Switzerland) [Molecules] 2022 Mar 17; Vol. 27 (6). Date of Electronic Publication: 2022 Mar 17.
Typ publikacji:: Journal Article
MeSH Terms:: Friedreich Ataxia*/genetics
Friedreich Ataxia*/metabolism
Iron-Binding Proteins*/genetics
Iron-Binding Proteins*/metabolism
Humans ; Protein Unfolding ; Thermodynamics ; Frataxin

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Skocz do pozycji: 6.

Tytuł:: Future Prospects of Gene Therapy for Friedreich's Ataxia.
Autorzy:: Ocana-Santero G; Centro de Biología Molecular Severo Ochoa (CSIC-UAM), Departamento de Biología Molecular, Universidad Autónoma de Madrid, Nicolás Cabrera 1, 28049 Madrid, Spain.; Department of Physiology, Anatomy and Genetics, Sherrington Building, Parks Road, University of Oxford, Oxford OX1 3PT, UK.
Díaz-Nido J; Centro de Biología Molecular Severo Ochoa (CSIC-UAM), Departamento de Biología Molecular, Universidad Autónoma de Madrid, Nicolás Cabrera 1, 28049 Madrid, Spain.
Herranz-Martín S; Centro de Biología Molecular Severo Ochoa (CSIC-UAM), Departamento de Biología Molecular, Universidad Autónoma de Madrid, Nicolás Cabrera 1, 28049 Madrid, Spain.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2021 Feb 11; Vol. 22 (4). Date of Electronic Publication: 2021 Feb 11.
Typ publikacji:: Journal Article; Review
MeSH Terms:: Friedreich Ataxia*/genetics
Friedreich Ataxia*/metabolism
Friedreich Ataxia*/therapy
Genetic Therapy*
Iron-Binding Proteins*/biosynthesis
Iron-Binding Proteins*/genetics
Animals ; Disease Models, Animal ; Humans ; Mice ; Frataxin

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Skocz do pozycji: 7.

Tytuł:: Molecular Details of the Frataxin-Scaffold Interaction during Mitochondrial Fe-S Cluster Assembly.
Autorzy:: Campbell CJ; Department of Pharmaceutical Sciences, Wayne State University, Detroit, MI 48201, USA.
Pall AE; Department of Pharmaceutical Sciences, Wayne State University, Detroit, MI 48201, USA.
Naik AR; Department of Pharmaceutical Sciences, Wayne State University, Detroit, MI 48201, USA.
Thompson LN; Department of Pharmaceutical Sciences, Wayne State University, Detroit, MI 48201, USA.
Stemmler TL; Department of Pharmaceutical Sciences, Wayne State University, Detroit, MI 48201, USA.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2021 Jun 02; Vol. 22 (11). Date of Electronic Publication: 2021 Jun 02.
Typ publikacji:: Journal Article; Review
MeSH Terms:: Iron/*metabolism
Iron-Binding Proteins/*genetics
Iron-Sulfur Proteins/*genetics
Sulfur/*metabolism
Carbon-Sulfur Lyases/genetics ; Humans ; Iron-Binding Proteins/metabolism ; Mitochondria/genetics ; Mitochondria/metabolism ; Multiprotein Complexes/genetics ; Multiprotein Complexes/ultrastructure ; Protein Binding/genetics ; Frataxin

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Skocz do pozycji: 8.

Tytuł:: Some Dietary Phenolic Compounds Can Activate Thyroid Peroxidase and Inhibit Lipoxygenase-Preliminary Study in the Model Systems.
Autorzy:: Habza-Kowalska E; Department of Biochemistry and Food Chemistry, University of Life Sciences, Skromna Str. 8, 20-704 Lublin, Poland.
Kaczor AA; Department of Synthesis and Chemical Technology of Pharmaceutical Substances with Computer Modeling Laboratory, Faculty of Pharmacy, Medical University of Lublin, 4A Chodzki St., 20-093 Lublin, Poland.; School of Pharmacy, University of Eastern Finland, Yliopistonranta 1, P.O. Box 1627, FI-70211 Kuopio, Finland.
Bartuzi D; Department of Synthesis and Chemical Technology of Pharmaceutical Substances with Computer Modeling Laboratory, Faculty of Pharmacy, Medical University of Lublin, 4A Chodzki St., 20-093 Lublin, Poland.
Piłat J; Department of General Surgery, Transplantology and Clinical Nutrition, Medical University of Lublin, Jaczewskiego Str. 8, 20-090 Lublin, Poland.
Gawlik-Dziki U; Department of Biochemistry and Food Chemistry, University of Life Sciences, Skromna Str. 8, 20-704 Lublin, Poland.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2021 May 12; Vol. 22 (10). Date of Electronic Publication: 2021 May 12.
Typ publikacji:: Journal Article
MeSH Terms:: Autoantigens/*metabolism
Enzyme Activators/*pharmacology
Iodide Peroxidase/*metabolism
Iron-Binding Proteins/*metabolism
Lipoxygenase Inhibitors/*pharmacology
Phytochemicals/*pharmacology
Protein-Lysine 6-Oxidase/*metabolism
Autoantigens/chemistry ; Cinnamates/chemistry ; Cinnamates/pharmacology ; Coumaric Acids/chemistry ; Coumaric Acids/pharmacology ; Dose-Response Relationship, Drug ; Enzyme Activators/chemistry ; Gallic Acid/analogs & derivatives ; Gallic Acid/chemistry ; Gallic Acid/pharmacology ; Humans ; Inhibitory Concentration 50 ; Iodide Peroxidase/chemistry ; Iron-Binding Proteins/chemistry ; Lipoxygenase Inhibitors/chemistry ; Models, Molecular ; Phytochemicals/chemistry ; Protein-Lysine 6-Oxidase/chemistry ; Structure-Activity Relationship

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Skocz do pozycji: 9.

Tytuł:: Drp1-dependent peptide reverse mitochondrial fragmentation, a homeostatic response in Friedreich ataxia.
Autorzy:: Johnson J; Department of Pediatrics and Neurology, University of Pennsylvania, Philadelphia, PA, USA.
Mercado-Ayón E; Department of Pediatrics and Neurology, University of Pennsylvania, Philadelphia, PA, USA.
Clark E; Department of Pediatrics and Neurology, University of Pennsylvania, Philadelphia, PA, USA.
Lynch D; Department of Pediatrics and Neurology, University of Pennsylvania, Philadelphia, PA, USA.; Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Lin H; Department of Pediatrics and Neurology, University of Pennsylvania, Philadelphia, PA, USA.; Children's Hospital of Philadelphia, Philadelphia, PA, USA.; Pokaż więcej
Źródło:: Pharmacology research & perspectives [Pharmacol Res Perspect] 2021 May; Vol. 9 (3), pp. e00755.
Typ publikacji:: Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:: Cell-Penetrating Peptides/*administration & dosage
Dynamins/*antagonists & inhibitors
Fibroblasts/*metabolism
Friedreich Ataxia/*metabolism
GTP Phosphohydrolases/*administration & dosage
Iron-Binding Proteins/*metabolism
Mitochondria/*metabolism
Oligopeptides/*administration & dosage
Peptide Fragments/*administration & dosage
Adenosine Triphosphate/metabolism ; Biomarkers ; Cells, Cultured ; Dynamins/metabolism ; Energy Metabolism ; Homeostasis ; Humans ; Iron-Binding Proteins/genetics ; RNA, Small Interfering/genetics ; Frataxin

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Skocz do pozycji: 10.

Tytuł:: Determination of the iron bioavailability, conformation, and rheology of iron-binding proteins from Tegillarca granosa.
Autorzy:: Li G; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.; Key Laboratory of Animal Protein Food Deep Processing Technology of Zhejiang Province, Ningbo University, Ningbo, China.
Zhan J; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Xu A; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Tan B; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Sun N; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Wang C; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Jia R; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Li C; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Zhang J; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.
Yang W; College of Food and Pharmaceutical Sciences, Ningbo University, Ningbo, China.; Key Laboratory of Animal Protein Food Deep Processing Technology of Zhejiang Province, Ningbo University, Ningbo, China.; Pokaż więcej
Źródło:: Journal of food biochemistry [J Food Biochem] 2021 Jan; Vol. 45 (1), pp. e13517. Date of Electronic Publication: 2020 Oct 28.
Typ publikacji:: Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:: Iron*
Iron-Binding Proteins*
Animals ; Biological Availability ; Bivalvia ; Caco-2 Cells ; Cattle ; Humans ; Rheology

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Skocz do pozycji: 11.

Tytuł:: Stress-dependent conformational changes of artemin: Effects of heat and oxidant.
Autorzy:: Takalloo Z; Department of Biochemistry, Faculty of Biological Sciences, Tarbiat Modares University, Tehran, Iran.
Ardakani ZA; Department of Biochemistry, Faculty of Biological Sciences, Tarbiat Modares University, Tehran, Iran.
Maroufi B; Mah Behin Gene Gostaran Company, Tehran, Iran.
Shahangian SS; Department of Biology, Faculty of Sciences, University of Guilan, Rasht, Iran.
Sajedi RH; Department of Biochemistry, Faculty of Biological Sciences, Tarbiat Modares University, Tehran, Iran.; Pokaż więcej
Źródło:: PloS one [PLoS One] 2020 Nov 16; Vol. 15 (11), pp. e0242206. Date of Electronic Publication: 2020 Nov 16 (Print Publication: 2020).
Typ publikacji:: Journal Article
MeSH Terms:: Arthropod Proteins/*chemistry
Iron-Binding Proteins/*chemistry
Oxidants/*chemistry
RNA-Binding Proteins/*chemistry
Animals ; Artemia/metabolism ; Arthropod Proteins/genetics ; Arthropod Proteins/metabolism ; Dimerization ; Electrophoresis, Polyacrylamide Gel ; Hydrogen Peroxide/chemistry ; Hydrophobic and Hydrophilic Interactions ; Iron-Binding Proteins/genetics ; Iron-Binding Proteins/metabolism ; Protein Structure, Tertiary ; RNA-Binding Proteins/genetics ; RNA-Binding Proteins/metabolism ; Recombinant Proteins/biosynthesis ; Recombinant Proteins/chemistry ; Recombinant Proteins/isolation & purification ; Spectrometry, Fluorescence ; Temperature

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Skocz do pozycji: 12.

Tytuł:: A Highly Conserved Iron-Sulfur Cluster Assembly Machinery between Humans and Amoeba Dictyostelium discoideum : The Characterization of Frataxin.
Autorzy:: Olmos J; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.
Pignataro MF; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.
Benítez Dos Santos AB; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.
Bringas M; Departamento de Química Inorgánica, Analítica y Química Física, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Instituto de Química Física de los Materiales, Medio Ambiente y Energía (INQUIMAE CONICET), Buenos Aires C1428EGA, Argentina.
Klinke S; Fundación Instituto Leloir, IIBBA-CONICET, and Plataforma Argentina de Biología Estructural y Metabolómica PLABEM, Av. Patricias Argentinas 435, Buenos Aires C1405BWE, Argentina.
Kamenetzky L; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.; IMPaM, CONICET, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires C1121ABG, Argentina.
Velazquez F; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.; Instituto de Química Biológica de la Facultad de Ciencias Exactas y Naturales (IQUIBICEN)-(UBA/CONICET), Buenos Aires C1428EGA, Argentina.
Santos J; Instituto de Biociencias, Biotecnología y Biología Traslacional (iB3), Departamento de Fisiología y Biología Molecular y Celular, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.; Consejo Nacional de Investigaciones Científicas y Técnicas, Rivadavia 1917, Buenos Aires C1033AAJ, Argentina.; Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires. Intendente Güiraldes 2160, Ciudad Universitaria, Buenos Aires C1428EGA, Argentina.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2020 Sep 17; Vol. 21 (18). Date of Electronic Publication: 2020 Sep 17.
Typ publikacji:: Journal Article
MeSH Terms:: Dictyostelium/*metabolism
Friedreich Ataxia/*genetics
Iron-Binding Proteins/*chemistry
Iron-Binding Proteins/*metabolism
Iron-Sulfur Proteins/*metabolism
Amino Acid Sequence/genetics ; Chromatography, High Pressure Liquid ; Circular Dichroism ; Computational Biology ; Crystallography ; Dictyostelium/genetics ; Humans ; Iron-Binding Proteins/genetics ; Iron-Sulfur Proteins/biosynthesis ; Iron-Sulfur Proteins/chemistry ; Iron-Sulfur Proteins/genetics ; Kinetics ; Molecular Dynamics Simulation ; Phylogeny ; Protein Binding ; Protein Structure, Tertiary ; Recombinant Proteins ; Sequence Alignment ; Spectrometry, Fluorescence ; Spectrophotometry, Ultraviolet ; Frataxin

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Skocz do pozycji: 13.

Tytuł:: Drosophila melanogaster frataxin: protein crystal and predicted solution structure with identification of the iron-binding regions.
Autorzy:: Rodrigues AV; Department of Biochemistry and Molecular Biology, Wayne State University, Detroit, Michigan, USA.
Batelu S; Department of Pharmaceutical Sciences, Wayne State University, Detroit, Michigan, USA.
Hinton TV; Department of Pharmaceutical Sciences, Wayne State University, Detroit, Michigan, USA.
Rotondo J; Department of Biochemistry and Molecular Biology, Wayne State University, Detroit, Michigan, USA.
Thompson L; Department of Pharmaceutical Sciences, Wayne State University, Detroit, Michigan, USA.
Brunzelle JS; Northwestern Synchrotron Research Centers, Life Science Collaborative Access Team, Northwestern University, Evanston, Illinois, USA.
Stemmler TL; Department of Biochemistry and Molecular Biology, Wayne State University, Detroit, Michigan, USA.; Pokaż więcej
Źródło:: Acta crystallographica. Section D, Structural biology [Acta Crystallogr D Struct Biol] 2023 Jan 01; Vol. 79 (Pt 1), pp. 22-30. Date of Electronic Publication: 2023 Jan 01.
Typ publikacji:: Journal Article
MeSH Terms:: Drosophila melanogaster*/metabolism
Neurodegenerative Diseases*
Animals ; Humans ; Iron-Binding Proteins/chemistry ; Iron-Binding Proteins/metabolism ; Saccharomyces cerevisiae/metabolism ; Iron/metabolism ; Sulfur/metabolism ; Frataxin

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Skocz do pozycji: 14.

Tytuł:: Extra-mitochondrial mouse frataxin and its implications for mouse models of Friedreich's ataxia.
Autorzy:: Weng L; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Laboureur L; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Wang Q; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Guo L; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Xu P; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Gottlieb L; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Lynch DR; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Departments of Pediatrics and Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Mesaros C; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA.; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA.
Blair IA; Penn Medicine/CHOP Center of Excellence in Friedreich's Ataxia, University of Pennsylvania, Philadelphia, PA, 19104, USA. .; Center of Excellence in Environmental Toxicology, Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, USA. .; Pokaż więcej
Źródło:: Scientific reports [Sci Rep] 2020 Sep 25; Vol. 10 (1), pp. 15788. Date of Electronic Publication: 2020 Sep 25.
Typ publikacji:: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms:: Disease Models, Animal*
Friedreich Ataxia/*pathology
Iron-Binding Proteins/*metabolism
Mitochondria/*metabolism
Amino Acid Sequence ; Animals ; Brain/metabolism ; Friedreich Ataxia/genetics ; Friedreich Ataxia/metabolism ; Heart/physiology ; Humans ; Iron-Binding Proteins/genetics ; Liver/metabolism ; Mice ; Mitochondria/genetics ; Frataxin

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Skocz do pozycji: 15.

Tytuł:: Increased airway iron parameters and risk for exacerbation in COPD: an analysis from SPIROMICS.
Autorzy:: Zhang WZ; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; New York-Presbyterian Hospital, New York, New York, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Oromendia C; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; Department of Population Health Sciences, Division of Biostatistics, Weill Cornell Medicine, New York, New York, USA.
Kikkers SA; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.
Butler JJ; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.
O'Beirne S; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; Department of Genetic Medicine, Weill Cornell Medicine, New York, New York, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Kim K; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.
O'Neal WK; University of North Carolina Marsico Lung Institute, Chapel Hill, North Carolina, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Freeman CM; Pulmonary and Critical Care Medicine Division, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, USA.; Veterans Affairs Ann Arbor Healthcare System, Ann Arbor, Michigan, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Christenson SA; University of California at San Francisco, San Francisco, California, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Peters SP; Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Wells JM; Division of Pulmonary, Allergy and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, Alabama, UK.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Doerschuk C; University of North Carolina Marsico Lung Institute, Chapel Hill, North Carolina, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Putcha N; Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Barjaktarevic I; Division of Pulmonary and Critical Care Medicine, University of California Los Angeles Medical Center, Los Angeles, California, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Woodruff PG; University of California at San Francisco, San Francisco, California, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Cooper CB; Division of Pulmonary and Critical Care Medicine, University of California Los Angeles Medical Center, Los Angeles, California, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Bowler RP; University of Colorado School of Medicine, Aurora, Colorado, USA.; National Jewish Health, Denver, Colorado, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Comellas AP; Division of Pulmonary and Critical Care, University of Iowa, Iowa City, Iowa, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Criner GJ; Department of Pulmonary & Critical Care Medicine, Temple University, Philadelphia, Pennsylvania, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Paine R 3rd; Section of Pulmonary and Critical Care Medicine, Salt Lake City Department of Veterans Affairs Medical Center, Salt Lake City, Utah, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Hansel NN; Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Han MK; Pulmonary and Critical Care Medicine Division, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Crystal RG; Department of Genetic Medicine, Weill Cornell Medicine, New York, New York, USA.
Kaner RJ; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; Department of Genetic Medicine, Weill Cornell Medicine, New York, New York, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Ballman KV; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; Department of Population Health Sciences, Division of Biostatistics, Weill Cornell Medicine, New York, New York, USA.
Curtis JL; Pulmonary and Critical Care Medicine Division, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, USA.; Veterans Affairs Ann Arbor Healthcare System, Ann Arbor, Michigan, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Martinez FJ; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA.; New York-Presbyterian Hospital, New York, New York, USA.; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA.
Cloonan SM; Joan and Sanford I. Weill Department of Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medicine, New York, USA. .; School of Medicine, Trinity Biomedical Sciences Institute and Tallaght University Hospital, Trinity College Dublin, Trinity, Ireland. .; SPIROMICS investigators, Collaborative Studies Coordinating Center, Department of Biostatistics Gillings School of Global Public Health, University of North Carolina at Chapel Hill 123 W. Franklin Street Suite 450, Chapel Hill, NC 27516, USA. .; Pokaż więcej
Źródło:: Scientific reports [Sci Rep] 2020 Jun 29; Vol. 10 (1), pp. 10562. Date of Electronic Publication: 2020 Jun 29.
Typ publikacji:: Clinical Trial; Journal Article; Multicenter Study; Observational Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:: Iron/*metabolism
Iron-Binding Proteins/*metabolism
Pulmonary Disease, Chronic Obstructive/*physiopathology
Adult ; Aged ; Aged, 80 and over ; Biomarkers/blood ; Bronchoalveolar Lavage Fluid/chemistry ; Disease Progression ; Female ; Ferritins/metabolism ; Forced Expiratory Volume ; Humans ; Iron/physiology ; Iron-Binding Proteins/physiology ; Lung/metabolism ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive/metabolism ; Respiratory Function Tests ; Risk Factors ; Severity of Illness Index

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Skocz do pozycji: 16.

Tytuł:: Dimethyl fumarate dosing in humans increases frataxin expression: A potential therapy for Friedreich's Ataxia.
Autorzy:: Jasoliya M; Department of Molecular Biosciences, School of Veterinary Medicine, University of California, Davis, California, United States of America.
Sacca F; Department of Neurosciences, Odontostomatological and Reproductive Sciences, University Federico II, Naples, Italy.
Sahdeo S; Department of Molecular Biosciences, School of Veterinary Medicine, University of California, Davis, California, United States of America.
Chedin F; Department of Molecular and Cellular Biology, University of California, Davis, California, United States of America.
Pane C; Department of Neurosciences, Odontostomatological and Reproductive Sciences, University Federico II, Naples, Italy.
Brescia Morra V; Department of Neurosciences, Odontostomatological and Reproductive Sciences, University Federico II, Naples, Italy.
Filla A; Department of Neurosciences, Odontostomatological and Reproductive Sciences, University Federico II, Naples, Italy.
Pook M; Department of Life Sciences, College of Health & Life Sciences, Brunel University London, Uxbridge, United Kingdom.
Cortopassi G; Department of Molecular Biosciences, School of Veterinary Medicine, University of California, Davis, California, United States of America.; Pokaż więcej
Źródło:: PloS one [PLoS One] 2019 Jun 03; Vol. 14 (6), pp. e0217776. Date of Electronic Publication: 2019 Jun 03 (Print Publication: 2019).
Typ publikacji:: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:: Dimethyl Fumarate/*therapeutic use
Friedreich Ataxia/*drug therapy
Iron-Binding Proteins/*metabolism
Animals ; Dimethyl Fumarate/pharmacology ; Disease Models, Animal ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; Friedreich Ataxia/blood ; Humans ; Iron-Binding Proteins/blood ; Iron-Binding Proteins/genetics ; Lymphocytes/drug effects ; Lymphocytes/metabolism ; Mice ; Models, Biological ; Mutation/genetics ; Organelle Biogenesis ; Transcription Initiation, Genetic ; Frataxin

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Skocz do pozycji: 17.

Tytuł:: A new tool to determine the cellular metabolic landscape: nanotechnology to the study of Friedreich's ataxia.
Autorzy:: Vannocci T; UK Dementia Research Institute at King's College London, London, SE5 9RT, United Kingdom.; The Wohl Institute at King's College London, London, SE5 9RT, United Kingdom.
Dinarelli S; Istituto di Struttura della Materia - CNR, Via del Fosso del Cavaliere 100, 00133, Rome, Italy.
Girasole M; Istituto di Struttura della Materia - CNR, Via del Fosso del Cavaliere 100, 00133, Rome, Italy.
Pastore A; UK Dementia Research Institute at King's College London, London, SE5 9RT, United Kingdom. .; The Wohl Institute at King's College London, London, SE5 9RT, United Kingdom. .
Longo G; Istituto di Struttura della Materia - CNR, Via del Fosso del Cavaliere 100, 00133, Rome, Italy. .; Pokaż więcej
Źródło:: Scientific reports [Sci Rep] 2019 Dec 17; Vol. 9 (1), pp. 19282. Date of Electronic Publication: 2019 Dec 17.
Typ publikacji:: Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:: Biosensing Techniques*
Motion*
Friedreich Ataxia/*diagnosis
Iron-Binding Proteins/*genetics
Friedreich Ataxia/genetics ; Friedreich Ataxia/metabolism ; Gene Expression Regulation/genetics ; HEK293 Cells ; Humans ; Iron-Binding Proteins/metabolism ; Nanotechnology/trends ; Oxidative Stress/genetics ; Frataxin

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Skocz do pozycji: 18.

Tytuł:: Neuroinflammation in Friedreich's Ataxia.
Autorzy:: Apolloni S; Department of Biology, Tor Vergata University of Rome, 00133 Roma, Italy.
Milani M; Department of Biology, Tor Vergata University of Rome, 00133 Roma, Italy.
D'Ambrosi N; Department of Biology, Tor Vergata University of Rome, 00133 Roma, Italy.; Pokaż więcej
Źródło:: International journal of molecular sciences [Int J Mol Sci] 2022 Jun 04; Vol. 23 (11). Date of Electronic Publication: 2022 Jun 04.
Typ publikacji:: Journal Article; Review
MeSH Terms:: Friedreich Ataxia*/metabolism
Animals ; Humans ; Iron/metabolism ; Iron-Binding Proteins/genetics ; Iron-Binding Proteins/metabolism ; Mitochondria/metabolism ; Neuroglia/metabolism ; Neuroinflammatory Diseases

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Skocz do pozycji: 19.

Tytuł:: Structural and functional characterization of a frataxin from a thermophilic organism.
Autorzy:: Rasheed M; King's College London, UK.; UK Dementia Research Institute at King's College London, UK.
Jamshidiha M; Department of Life Sciences, Imperial College, London, South Kensington, UK.
Puglisi R; King's College London, UK.; UK Dementia Research Institute at King's College London, UK.
Yan R; King's College London, UK.; UK Dementia Research Institute at King's College London, UK.
Cota E; Department of Life Sciences, Imperial College, London, South Kensington, UK.
Pastore A; King's College London, UK.; UK Dementia Research Institute at King's College London, UK.; University of Pavia, Italy.; Pokaż więcej
Źródło:: The FEBS journal [FEBS J] 2019 Feb; Vol. 286 (3), pp. 495-506. Date of Electronic Publication: 2019 Jan 30.
Typ publikacji:: Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:: Carbon-Sulfur Lyases/*chemistry
Chaetomium/*chemistry
Escherichia coli Proteins/*chemistry
Fungal Proteins/*chemistry
Iron/*chemistry
Iron-Binding Proteins/*chemistry
Iron-Sulfur Proteins/*chemistry
Amino Acid Sequence ; Binding Sites ; Carbon-Sulfur Lyases/genetics ; Carbon-Sulfur Lyases/metabolism ; Chaetomium/genetics ; Chaetomium/metabolism ; Cloning, Molecular ; Crystallography, X-Ray ; Escherichia coli/genetics ; Escherichia coli/metabolism ; Escherichia coli Proteins/genetics ; Escherichia coli Proteins/metabolism ; Fungal Proteins/genetics ; Fungal Proteins/metabolism ; Gene Expression ; Humans ; Iron/metabolism ; Iron-Binding Proteins/genetics ; Iron-Binding Proteins/metabolism ; Iron-Sulfur Proteins/genetics ; Iron-Sulfur Proteins/metabolism ; Kinetics ; Models, Molecular ; Protein Binding ; Protein Conformation, alpha-Helical ; Protein Conformation, beta-Strand ; Protein Folding ; Protein Interaction Domains and Motifs ; Protein Stability ; Recombinant Proteins/chemistry ; Recombinant Proteins/genetics ; Recombinant Proteins/metabolism ; Sequence Alignment ; Sequence Homology, Amino Acid ; Species Specificity ; Static Electricity ; Thermodynamics ; Frataxin

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Skocz do pozycji: 20.

Tytuł:: Characterization of a new N-terminally acetylated extra-mitochondrial isoform of frataxin in human erythrocytes.
Autorzy:: Guo L; Penn SRP Center and Center of Excellence in Environmental Toxicology Center, Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.
Wang Q; Penn SRP Center and Center of Excellence in Environmental Toxicology Center, Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.
Weng L; Penn SRP Center and Center of Excellence in Environmental Toxicology Center, Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.
Hauser LA; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.
Strawser CJ; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.
Mesaros C; Penn SRP Center and Center of Excellence in Environmental Toxicology Center, Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.
Lynch DR; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, 19104, United States.; Departments of Pediatrics and Neurology Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States.
Blair IA; Penn SRP Center and Center of Excellence in Environmental Toxicology Center, Department of Systems Pharmacology and Translational Therapeutics Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104, United States. .; Penn/CHOP Center of Excellence in Friedreich's ataxia, Philadelphia, PA, 19104, United States. .; Pokaż więcej
Źródło:: Scientific reports [Sci Rep] 2018 Nov 19; Vol. 8 (1), pp. 17043. Date of Electronic Publication: 2018 Nov 19.
Typ publikacji:: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:: Erythrocytes/*metabolism
Iron-Binding Proteins/*blood
Protein Isoforms/*blood
Acetylation ; Amino Acid Sequence ; HEK293 Cells ; Humans ; Iron-Binding Proteins/chemistry ; Iron-Binding Proteins/genetics ; Mitochondria/metabolism ; Protein Isoforms/chemistry ; Protein Isoforms/genetics ; RNA, Messenger/genetics ; Frataxin

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