المؤلفون: Bondue T; Laboratory of Pediatric Nephrology, Department of Development and Regeneration, KU Leuven, Leuven, Belgium., Khodaparast L; Switch Laboratory, VIB Center for Brain and Disease Research, Leuven, Belgium.; Switch Laboratory, Department of Cellular and Molecular Medicine, KU Leuven, Leuven, Belgium., Khodaparast L; Switch Laboratory, VIB Center for Brain and Disease Research, Leuven, Belgium.; Switch Laboratory, Department of Cellular and Molecular Medicine, KU Leuven, Leuven, Belgium., Cairoli S; Laboratory of Metabolic Biochemistry, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy., Goffredo BM; Laboratory of Metabolic Biochemistry, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy., Gijsbers R; Laboratory for Molecular Virology and Gene Therapy, Department of Pharmaceutical and Pharmacological Sciences, KU Leuven, Leuven, Belgium.; Leuven Viral Vector Core, KU Leuven, Leuven, Belgium., van den Heuvel L; Laboratory of Pediatric Nephrology, Department of Development and Regeneration, KU Leuven, Leuven, Belgium.; Department of Pediatric Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands., Levtchenko E; Laboratory of Pediatric Nephrology, Department of Development and Regeneration, KU Leuven, Leuven, Belgium.; Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam UMC, Amsterdam, The Netherlands.

المصدر: American journal of physiology. Renal physiology [Am J Physiol Renal Physiol] 2024 Jun 01; Vol. 326 (6), pp. F981-F987. Date of Electronic Publication: 2024 Mar 28.

نوع المنشور: Journal Article

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901990 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-1466 (Electronic) Linking ISSN: 15221466 NLM ISO Abbreviation: Am J Physiol Renal Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Zebrafish*/metabolism , Kidney Tubules, Proximal*/metabolism , Kidney Tubules, Proximal*/pathology , Cystinosis*/metabolism , Cystinosis*/genetics , Cystinosis*/pathology , Cystine*/metabolism , Disease Models, Animal*, Animals ; Humans ; Zebrafish Proteins/metabolism ; Zebrafish Proteins/genetics ; Epithelial Cells/metabolism ; Amino Acid Transport Systems, Neutral/genetics ; Amino Acid Transport Systems, Neutral/metabolism ; CRISPR-Cas Systems

المؤلفون: Hollywood JA; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Kallingappa PK; Vernon Jansen Unit, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand., Cheung PY; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Martis RM; Department of Physiology, The University of Auckland, Auckland, New Zealand., Sreebhavan S; Auckland Cancer Society Research Centre, The University of Auckland, Auckland, New Zealand., 'Atiola RD; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Chatterjee A; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Buckels EJ; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Matthews BG; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Lewis PM; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand., Davidson AJ; Department of Molecular Medicine and Pathology, The University of Auckland, Auckland, New Zealand.

المصدر: American journal of physiology. Renal physiology [Am J Physiol Renal Physiol] 2022 Aug 01; Vol. 323 (2), pp. F156-F170. Date of Electronic Publication: 2022 Jun 13.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901990 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-1466 (Electronic) Linking ISSN: 15221466 NLM ISO Abbreviation: Am J Physiol Renal Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Amino Acid Transport Systems, Neutral*/genetics , Amino Acid Transport Systems, Neutral*/metabolism , Cystinosis*/drug therapy , Cystinosis*/genetics , Cystinosis*/metabolism , Fanconi Syndrome*/genetics, Animals ; Cysteamine/pharmacology ; Cysteamine/therapeutic use ; Cystine/genetics ; Cystine/metabolism ; Cystine/therapeutic use ; Phenotype ; Rats

المؤلفون: Anderson CL; Department of Medicine/Pulmonary, Emory University School of Medicine, 615 Michael Street, Atlanta, GA 30322, USA., Iyer SS, Ziegler TR, Jones DP

المصدر: American journal of physiology. Regulatory, integrative and comparative physiology [Am J Physiol Regul Integr Comp Physiol] 2007 Sep; Vol. 293 (3), pp. R1069-75. Date of Electronic Publication: 2007 Jun 13.

نوع المنشور: Journal Article; Research Support, N.I.H., Extramural

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901230 Publication Model: Print-Electronic Cited Medium: Print ISSN: 0363-6119 (Print) Linking ISSN: 03636119 NLM ISO Abbreviation: Am J Physiol Regul Integr Comp Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Cysteine/*metabolism , Cystine/*metabolism , Glutathione/*metabolism, Biological Transport, Active/drug effects ; Buthionine Sulfoximine/pharmacology ; Culture Media ; Cysteine/deficiency ; Cystine/deficiency ; Disulfides/metabolism ; Extracellular Space/metabolism ; HT29 Cells ; Humans ; Kinetics ; Oxidation-Reduction ; Oxidative Stress/drug effects ; Sulfhydryl Compounds/metabolism

المؤلفون: Buckley BJ; Departments of Medicine and Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina 27710, USA. barbara.buckley@duke.edu, Whorton AR

المصدر: American journal of physiology. Cell physiology [Am J Physiol Cell Physiol] 2000 Oct; Vol. 279 (4), pp. C1168-76.

نوع المنشور: Journal Article; Research Support, U.S. Gov't, P.H.S.

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901225 Publication Model: Print Cited Medium: Print ISSN: 0363-6143 (Print) Linking ISSN: 03636143 NLM ISO Abbreviation: Am J Physiol Cell Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Cystine/*pharmacokinetics , Endothelium, Vascular/*metabolism , Glutathione/*metabolism , Molsidomine/*analogs & derivatives , Muscle, Smooth, Vascular/*metabolism , Nitrates/*pharmacology , Penicillamine/*analogs & derivatives, Adaptation, Physiological/physiology ; Animals ; Cattle ; Cells, Cultured ; Cystine/metabolism ; Dose-Response Relationship, Drug ; Endothelium, Vascular/cytology ; Endothelium, Vascular/drug effects ; Free Radical Scavengers/pharmacology ; Glutamic Acid/metabolism ; Glutamic Acid/pharmacology ; Metalloporphyrins/pharmacology ; Molsidomine/pharmacology ; Muscle, Smooth, Vascular/cytology ; Muscle, Smooth, Vascular/drug effects ; Nitric Oxide Donors/pharmacology ; Nucleic Acid Synthesis Inhibitors/pharmacology ; Penicillamine/pharmacology ; Protein Synthesis Inhibitors/pharmacology ; S-Nitroso-N-Acetylpenicillamine ; Sodium/metabolism ; Sodium/pharmacology ; Superoxide Dismutase/metabolism

المؤلفون: Ben-Nun A; Department of Physiology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel., Bashan N, Potashnik R, Cohen-Luria R, Moran A

المصدر: The American journal of physiology [Am J Physiol] 1993 Dec; Vol. 265 (6 Pt 2), pp. F839-44.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 0370511 Publication Model: Print Cited Medium: Print ISSN: 0002-9513 (Print) Linking ISSN: 00029513 NLM ISO Abbreviation: Am J Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Cystine/*analogs & derivatives , Fanconi Syndrome/*chemically induced, Adenosine Triphosphate/metabolism ; Animals ; Cell Line ; Cell Membrane/metabolism ; Cystine/metabolism ; Cystine/pharmacology ; Fanconi Syndrome/physiopathology ; Kidney/metabolism ; Kidney/pathology ; Kidney/physiopathology ; Male ; Monosaccharide Transport Proteins/metabolism ; Phlorhizin/metabolism ; Rats ; Rats, Inbred Strains ; Sodium/pharmacology ; Sodium-Potassium-Exchanging ATPase/antagonists & inhibitors

المؤلفون: Ben-Nun A; Department of Physiology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel., Bashan N, Potashnik R, Cohen-Luria R, Moran A

المصدر: The American journal of physiology [Am J Physiol] 1992 Aug; Vol. 263 (2 Pt 1), pp. C516-20.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 0370511 Publication Model: Print Cited Medium: Print ISSN: 0002-9513 (Print) Linking ISSN: 00029513 NLM ISO Abbreviation: Am J Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Cystine/*analogs & derivatives , Kidney/*metabolism , Sodium/*physiology, Animals ; Biological Transport/drug effects ; Carrier Proteins/metabolism ; Cell Line ; Cystine/pharmacology ; Epithelial Cells ; Epithelium/metabolism ; Glucose/metabolism ; Intracellular Membranes/metabolism ; Kidney/cytology ; Potassium/metabolism ; Rubidium/metabolism ; Sodium/metabolism ; Sodium-Hydrogen Exchangers ; Sodium-Potassium-Exchanging ATPase/metabolism

المؤلفون: Coloso RM; Division of Nutritional Sciences, Cornell University, Ithaca, New York 14853., Drake MR, Stipanuk MH

المصدر: The American journal of physiology [Am J Physiol] 1990 Sep; Vol. 259 (3 Pt 1), pp. E443-50.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 0370511 Publication Model: Print Cited Medium: Print ISSN: 0002-9513 (Print) Linking ISSN: 00029513 NLM ISO Abbreviation: Am J Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Alkynes*, Chelating Agents/*pharmacology , Cysteine/*metabolism , Cystine/*metabolism , Liver/*metabolism , Phenanthrolines/*pharmacology, Animals ; Cells, Cultured ; Copper/metabolism ; Cysteine/pharmacology ; Cystine/pharmacology ; Glycine/analogs & derivatives ; Glycine/pharmacology ; Kinetics ; Liver/drug effects ; Male ; Models, Biological ; Pargyline/analogs & derivatives ; Pargyline/pharmacology ; Rats ; Rats, Inbred Strains

المؤلفون: Storch KJ; Laboratory of Human Nutrition, School of Science, Massachusetts Institute of Technology, Cambridge 02139., Wagner DA, Burke JF, Young VR

المصدر: The American journal of physiology [Am J Physiol] 1990 May; Vol. 258 (5 Pt 1), pp. E790-8.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 0370511 Publication Model: Print Cited Medium: Print ISSN: 0002-9513 (Print) Linking ISSN: 00029513 NLM ISO Abbreviation: Am J Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Cystine/*metabolism , Methionine/*metabolism, Adult ; Carbon Isotopes ; Cystine/blood ; Deuterium ; Diet ; Humans ; Isotope Labeling/methods ; Male ; Methionine/blood ; Radioisotope Dilution Technique

المؤلفون: Di Giacopo A; Institute of Physiology-Zurich Center for Integrative Human Physiology (ZIHP University of Zurich, Winterthurerstrasse 190, CH-8075 Zurich, Switzerland. wagnerca@access.uzh.ch., Rubio-Aliaga I, Cantone A, Artunc F, Rexhepaj R, Frey-Wagner I, Font-Llitjós M, Gehring N, Stange G, Jaenecke I, Mohebbi N, Closs EI, Palacín M, Nunes V, Daniel H, Lang F, Capasso G, Wagner CA

المصدر: American journal of physiology. Renal physiology [Am J Physiol Renal Physiol] 2013 Dec 15; Vol. 305 (12), pp. F1645-55. Date of Electronic Publication: 2013 Oct 09.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901990 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-1466 (Electronic) Linking ISSN: 15221466 NLM ISO Abbreviation: Am J Physiol Renal Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Amino Acid Transport Systems, Basic/*deficiency , Amino Acids, Diamino/*metabolism , Cystine/*metabolism , Cystinuria/*metabolism, Amino Acid Transport Systems, Basic/genetics ; Animals ; Cystinuria/physiopathology ; Disease Models, Animal ; Glomerular Filtration Rate/physiology ; Kidney/metabolism ; Kidney/physiopathology ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout

المؤلفون: Wang ZH; Key Laboratory of Stem Cell Biology and Laboratory of Molecular Cardiology, Institute of Health Sciences, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, and Shanghai Jiao Tong University School of Medicine, Shanghai, China., Chen YX, Zhang CM, Wu L, Yu Z, Cai XL, Guan Y, Zhou ZN, Yang HT

المصدر: American journal of physiology. Heart and circulatory physiology [Am J Physiol Heart Circ Physiol] 2011 Oct; Vol. 301 (4), pp. H1695-705. Date of Electronic Publication: 2011 Aug 05.

نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't

بيانات الدورية: Publisher: American Physiological Society Country of Publication: United States NLM ID: 100901228 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-1539 (Electronic) Linking ISSN: 03636135 NLM ISO Abbreviation: Am J Physiol Heart Circ Physiol Subsets: MEDLINE

مواضيع طبية MeSH: Myocardial Reperfusion*, Hypoxia/*physiopathology , Myocardial Contraction/*physiology , Myocardial Ischemia/*physiopathology , Myocardial Reperfusion Injury/*prevention & control , Signal Transduction/*physiology, Air Pressure ; Animals ; Antioxidants/pharmacology ; Cell Survival/physiology ; Cystine/analogs & derivatives ; Cystine/pharmacology ; Enzyme Activation/physiology ; KATP Channels/metabolism ; L-Lactate Dehydrogenase/metabolism ; Lipid Peroxidation ; Male ; Mitochondria, Heart/metabolism ; Mitochondria, Heart/physiology ; Myocardial Infarction/pathology ; Myocytes, Cardiac/physiology ; Oncogene Protein v-akt/metabolism ; Oncogene Protein v-akt/physiology ; Oxidation-Reduction ; Protein Kinase C-epsilon/metabolism ; Rats ; Rats, Sprague-Dawley ; Reactive Oxygen Species/metabolism ; Recovery of Function