يعرض 1 - 10 نتائج من 21 نتيجة بحث عن '"Hutchinson Gilford progeria syndrome"', وقت الاستعلام: 1.31s تنقيح النتائج
  1. 1
    دورية أكاديمية
    Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations.

    المؤلفون: Rivera-Torres, José, Calvo, Conrado J, Llach, Anna, Guzmán-Martínez, Gabriela, Caballero, Ricardo, González-Gómez, Cristina, Jiménez-Borreguero, Luis J, Guadix, Juan A, Osorio, Fernando G, López-Otín, Carlos, Herraiz-Martínez, Adela, Cabello, Nuria, Vallmitjana, Alex, Benítez, Raul, Gordon, Leslie B, Jalife, José, Pérez-Pomares, José M, Tamargo, Juan, Delpón, Eva, Hove-Madsen, Leif, Filgueiras-Rama, David, Andrés, Vicente

    مصطلحات موضوعية: Hutchinson–Gilford progeria syndrome, calcium handling, connexin43, prelamin A, progerin, Adolescent, Adult, Animals, Arrhythmias, Cardiac, Calcium, Cardiac Conduction System Disease, Child, Preschool, Connexin 43, Female, Heart, Humans, Male, Membrane Proteins, Metalloendopeptidases, Mice, Inbred C57BL, Knockout, Myocardium, Nuclear Lamina, Progeria, Sarcoplasmic Reticulum, Young Adult

    العلاقة: http://hdl.handle.net/10668/10568Test; PMC5135377; https://europepmc.org/articles/pmc5135377?pdf=renderTest; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5135377/pdfTest

    الإتاحة: https://doi.org/10.1073/pnas.1603754113Test
    http://hdl.handle.net/10668/10568Test
    https://europepmc.org/articles/pmc5135377?pdf=renderTest
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5135377/pdfTest

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  2. 2
    Paclitaxel mitigates structural alterations and cardiac conduction system defects in a mouse model of Hutchinson-Gilford progeria syndrome

    المؤلفون: Yaazan Blanco, María J. Andrés-Manzano, José Jalife, Víctor Fanjul, Pilar Gonzalo, David Filgueiras-Rama, Andre Monteiro da Rocha, Vicente Andrés, J Jaime Díaz-Larrosa, Cristina González-Gómez, Alvaro Macias, Andrew Allan, Daniela Ponce-Balbuena

    المساهمون: Ministerio de Ciencia e Innovación (España), Instituto de Salud Carlos III, Unión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF), Progeria Research Foundation, Ministerio de Ciencia e Innovación. Centro de Excelencia Severo Ochoa (España), Fundación ProCNIC, NIH - National Heart, Lung, and Blood Institute (NHLBI) (Estados Unidos)

    المصدر: Cardiovascular Research

    مصطلحات موضوعية: Male, Refractory Period, Electrophysiological, Physiology, Swine, Cardiac index, Action Potentials, Laminopathy, Afterdepolarization, Progerin, Progeria, Heart Rate, Tubulin, Myocytes, Cardiac, AcademicSubjects/MED00200, Cytoskeleton, Excitation Contraction Coupling, Cardiomyocytes, integumentary system, Lamin Type A, Electrophysiology, Cardiology, Swine, Miniature, Female, Electrical conduction system of the heart, Cardiology and Cardiovascular Medicine, Anti-Arrhythmia Agents, medicine.medical_specialty, Paclitaxel, Cardiac Electrophysiology and Arrhythmia, Heart Conduction System, Physiology (medical), Internal medicine, Animal model of cardiovascular disease, Cardiac conduction, medicine, Repolarization, Animals, Genetic Predisposition to Disease, Lamin A/C, Hutchinson–Gilford progeria syndrome, business.industry, Arrhythmias, Cardiac, Original Articles, medicine.disease, Mice, Mutant Strains, Disease Models, Animal, Mutation, business

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6f24170b70fb89a7a0130672f81004c0Test
    https://pubmed.ncbi.nlm.nih.gov/33624748Test


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  3. 3
    Ultrasonic Characteristics of Cardiovascular Changes in Children with Hutchinson–Gilford Progeria Syndrome: A Comparative Study with Normal Children and Aging People

    المؤلفون: Fan Yang, Minjuan Zheng, Hong-Ping Song, Dang-Jie Wu, Wen-Qing Gong, Ying Zhang, Xin Sun, Xiaoni Zhao

    المصدر: BioMed Research International, Vol 2020 (2020)
    BioMed Research International

    مصطلحات موضوعية: 0301 basic medicine, Male, medicine.medical_specialty, Aging, congenital, hereditary, and neonatal diseases and abnormalities, Longitudinal strain, Article Subject, Heart Ventricles, 030204 cardiovascular system & hematology, Pulse Wave Analysis, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, 0302 clinical medicine, Progeria, Internal medicine, medicine, Humans, Child, Aged, Ejection fraction, General Immunology and Microbiology, integumentary system, business.industry, Ultrasound, Infant, nutritional and metabolic diseases, Stroke Volume, General Medicine, Middle Aged, medicine.disease, 030104 developmental biology, medicine.anatomical_structure, Carotid Arteries, Ventricle, Child, Preschool, Normal children, Cardiology, Elasticity Imaging Techniques, Medicine, Female, Abnormality, business, Hutchinson Gilford Progeria Syndrome, Research Article

    وصف الملف: text/xhtml

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c9c592e62df5f2c4176dc4384c32fd98Test
    https://doaj.org/article/f1056ec66909400f964656e70b2c9190Test


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  4. 4
    Author Correction: Progerinin, an optimized progerin-lamin A binding inhibitor, ameliorates premature senescence phenotypes of Hutchinson-Gilford progeria syndrome

    المؤلفون: Jieun Kim, Soo Yong Lee, So-mi Kang, Jeong Su Kim, Bum-Joon Park, Tae Sung Hwang, Nam-Chul Ha, Soyoung Park, Min-Ho Yoon, Seock Yong Kang, Jinsook Ahn, Tae-Gyun Woo, Jeongmin Joo, So-Young Kim, Kyu Jin Chung, Gyu-Yong Song, Jung-Hyun Cho, So Yon An, Ah-Young Oh

    المصدر: Communications Biology, Vol 4, Iss 1, Pp 1-1 (2021)
    Communications Biology

    مصطلحات موضوعية: Senescence, Male, Adolescent, QH301-705.5, Primary Cell Culture, Drug Evaluation, Preclinical, Medicine (miscellaneous), General Biochemistry, Genetics and Molecular Biology, Mice, Progeria, Medicine, Animals, Humans, Biology (General), Author Correction, Child, business.industry, Drug discovery, Premature senescence, Progerin, Lamin Type A, Phenotype, Disease Models, Animal, HEK293 Cells, Cancer research, Female, General Agricultural and Biological Sciences, business, Hutchinson Gilford Progeria Syndrome, Lamin

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d67dd7878c23d025b86efe71ac12d92cTest
    https://doaj.org/article/6f0c7f74aeec45738af0d9e5e5954b00Test


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  5. 5
    Transient introduction of human telomerase mRNA improves hallmarks of progeria cells

    المؤلفون: Gang Zhou, Ning Zhang, Jerry W. Shay, Sei Sho, Enzo Tedone, Tsung Po Lai, John P. Cooke, Ivone G. Bruno, Yanhui Li

    المصدر: Aging Cell

    مصطلحات موضوعية: 0301 basic medicine, Male, Telomerase, Pyridines, LMNA, 0302 clinical medicine, Progeria, Piperidines, Enzyme Inhibitors, Child, Cellular Senescence, integumentary system, Telomere, Progerin, Lamin Type A, Cell biology, Child, Preschool, Female, Original Article, Senescence, Adult, congenital, hereditary, and neonatal diseases and abnormalities, Adolescent, RNA therapy, Biology, Transfection, telomerase, Cell Line, 03 medical and health sciences, Hutchinson‐Gilford progeria syndrome, medicine, Farnesyltranstransferase, Humans, Telomerase reverse transcriptase, RNA, Messenger, Aged, aging, Infant, Newborn, nutritional and metabolic diseases, Infant, Telomere Homeostasis, Cell Biology, Original Articles, Fibroblasts, medicine.disease, telomeres, 030104 developmental biology, 030217 neurology & neurosurgery, Lamin

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::db2a7073b3befb91dac3586d4981fd7aTest
    https://pubmed.ncbi.nlm.nih.gov/31152494Test


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  6. 6
    Targeting of NAT10 enhances healthspan in a mouse model of human accelerated aging syndrome

    المؤلفون: Balmus, Gabriel, Larrieu, Delphine, Barros, Ana C, Collins, Casey, Abrudan, Monica, Demir, Mukerrem, Geisler, Nicola J, Lelliott, Christopher J, White, Jacqueline K, Karp, Natasha A, Atkinson, James, Kirton, Andrea, Jacobsen, Matt, Clift, Dean, Rodriguez, Raphael, Sanger Mouse Genetics Project, Adams, David J, Jackson, Stephen P

    المساهمون: Balmus, Gabriel [0000-0003-2872-4468], Larrieu, Delphine [0000-0002-2335-9361], Collins, Casey [0000-0002-5333-1116], Abrudan, Monica [0000-0003-0228-4353], Lelliott, Christopher J [0000-0001-8087-4530], Atkinson, James [0000-0002-7979-4960], Jacobsen, Matt [0000-0002-2713-1188], Rodriguez, Raphael [0000-0001-7668-446X], Apollo - University of Cambridge Repository

    المصدر: Nature Communications, Vol 9, Iss 1, Pp 1-14 (2018)

    مصطلحات موضوعية: Male, congenital, hereditary, and neonatal diseases and abnormalities, premature ageing, Science, LMNA, Kaplan-Meier Estimate, Hutchinson-Gilford Progeria Syndrome, Genomic Instability, N-Terminal Acetyltransferases, Mice, Progeria, Animals, Humans, lcsh:Science, N-Terminal Acetyltransferase A, Mice, Knockout, integumentary system, Hydrazones, nutritional and metabolic diseases, Aging, Premature, Lamin Type A, NAT10, Mice, Inbred C57BL, Disease Models, Animal, Thiazoles, Female, lcsh:Q, DNA Damage

    وصف الملف: application/pdf

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4ed8f8bd77bbb0f7c0537defaa23ce66Test


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  7. 7
    Farnesyltransferase inhibitor and rapamycin correct aberrant genome organisation and decrease DNA damage respectively, in Hutchinson-Gilford progeria syndrome fibroblasts

    المؤلفون: Mehmet U. Bikkul, Joanna M. Bridger, Craig S. Clements, Lauren S. Godwin, Ian R. Kill, Martin W. Goldberg

    المصدر: Biogerontology
    Biogerontology, 2018, Vol.19(6), pp.579-602 [Peer Reviewed Journal]

    مصطلحات موضوعية: 0301 basic medicine, Aging, congenital, hereditary, and neonatal diseases and abnormalities, Biology, medicine.disease_cause, Progeroid syndromes, Cell Line, LMNA, 03 medical and health sciences, Progeria, Prenylation, Genome organisation, Farnesyltransferase inhibitors, medicine, Farnesyltranstransferase, Humans, Rapamycin, Insulin-Like Growth Factor I, Sirolimus, Mutation, Hutchinson–Gilford progeria syndrome, integumentary system, Diphosphonates, Genome, Human, Farnesyltransferase inhibitor, nutritional and metabolic diseases, Membrane Proteins, Metalloendopeptidases, Fibroblasts, medicine.disease, Progerin, Lamin Type A, Lamins, 030104 developmental biology, COMET assay, Cancer research, DNA damage, Drug Therapy, Combination, Female, Geriatrics and Gerontology, Hydroxymethylglutaryl-CoA Reductase Inhibitors, Gerontology, Protein Processing, Post-Translational, Lamin, Research Article

    وصف الملف: application/pdf

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::429d5898315377b4d14f0feadec93d82Test
    https://pubmed.ncbi.nlm.nih.gov/29907918Test


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  8. 8
    Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations

    المؤلفون: Juan Tamargo, Conrado J. Calvo, Raul Benitez, Ricardo Caballero, Gabriela Guzmán-Martínez, Nuria Cabello, Carlos López-Otín, David Filgueiras-Rama, Adela Herraiz-Martínez, Vicente Andrés, Fernando G. Osorio, Juan Antonio Guadix, Eva Delpón, José Rivera-Torres, José M. Pérez-Pomares, José Jalife, Leif Hove-Madsen, Luis Jesús Jiménez-Borreguero, Anna Llach, Cristina González-Gómez, Alexander Vallmitjana, Leslie B. Gordon

    المساهمون: Universitat Politècnica de Catalunya. Departament d'Enginyeria de Sistemes, Automàtica i Informàtica Industrial, Universitat Politècnica de Catalunya. SISBIO - Senyals i Sistemes Biomèdics, Ministerio de Economía y Competitividad (España), Unión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF), Instituto de Salud Carlos III, Unión Europea, Fundación Cajastur, Fundación ProCNIC

    المصدر: Rivera-Torres, José Calvo, Conrado J. Llach, Anna Guzmán-Martínez, Gabriela Caballero, Ricardo González-Gómez, Cristina Jiménez Borreguero, Luis J. Guadix, Juan A. Osorio, Fernando G. López-Otín, Carlos Herraiz-Martínez, Adela Cabello, Nuria Vallmitjana, Alex Benítez, Raul Gordon, Leslie B. Jalife, José Pérez-Pomares, José M. Tamargo, Juan Delpón, Eva Hove-Madsen, Leif Filgueiras-Rama, David Andrés, Vicente (2016) Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations Proceedings of the National Academy of Sciences of the United States of America
    PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
    r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
    instname
    Repositorio Institucional de la Consejería de Sanidad de la Comunidad de Madrid
    Consejería de Sanidad de la Comunidad de Madrid
    Scopus
    RUO. Repositorio Institucional de la Universidad de Oviedo
    RODERIC. Repositorio Institucional de la Universitat de Valéncia
    Repisalud
    Instituto de Salud Carlos III (ISCIII)
    Recercat. Dipósit de la Recerca de Catalunya
    UPCommons. Portal del coneixement obert de la UPC
    Universitat Politècnica de Catalunya (UPC)
    r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
    r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
    Fundació Sant Joan de Déu
    ABACUS. Repositorio de Producción Científica
    Universidad Europea (UEM)

    مصطلحات موضوعية: 0301 basic medicine, Male, Hutchinson–Gilford progeria syndrome, calcium handling, connexin43, prelamin A, progerin, Electrònica en cardiologia, 030204 cardiovascular system & hematology, Pathogenesis, Ciencias Biomedicas, 0302 clinical medicine, Progeria, Cardiac Conduction System Disease, fisiologia, patología, Tecnología médica, Child, Ciencias médicas, Mice, Knockout, prelamin A, Multidisciplinary, integumentary system, Metalloendopeptidases, Heart, Progerin, Hutchinson-Gilford progeria syndrome, 3. Good health, Enginyeria biomèdica::Electrònica biomèdica::Electrònica en cardiologia [Àrees temàtiques de la UPC], Sarcoplasmic Reticulum, medicine.anatomical_structure, PNAS Plus, Child, Preschool, cardiovascular system, Nuclear lamina, Female, medicine.symptom, Bradycardia, Adult, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Adolescent, Biology, 03 medical and health sciences, QRS complex, Young Adult, Electrònica mèdica, Internal medicine, medicine, Animals, Humans, PR interval, Hutchinson–Gilford progeria syndrome, Nuclear Lamina, Myocardium, Membrane Proteins, nutritional and metabolic diseases, Arrhythmias, Cardiac, medicine.disease, Medical electronics, connexin43, Mice, Inbred C57BL, 030104 developmental biology, Endocrinology, Ventricle, progerin, Connexin 43, calcium handling, sistema cardiovascular, Calcium

    وصف الملف: application/pdf

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::55c27ec0f47446705dd2edafa5b8957cTest
    https://hdl.handle.net/10550/70444Test


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  9. 9
    Dental and craniofacial characteristics in a patient with Hutchinson–Gilford progeria syndrome

    المؤلفون: Andreas Jäger, Werner Götz, Lina Gölz, Christoph Reichert, James Deschner, Michael Wolf

    المصدر: Journal of Orofacial Orthopedics / Fortschritte der Kieferorthopädie. 75:251-263

    مصطلحات موضوعية: Premature aging, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Tooth Movement Techniques, Dentistry, Orthodontics, Progeria, medicine, Humans, Initial treatment, Craniofacial, Child, Periodontitis, integumentary system, business.industry, nutritional and metabolic diseases, medicine.disease, Combined Modality Therapy, Dermatology, Anti-Bacterial Agents, stomatognathic diseases, Treatment Outcome, Tooth Extraction, Oral and maxillofacial surgery, Female, Oral Surgery, business, Hutchinson Gilford Progeria Syndrome, Lamin

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::11b4746b92f2bf9d8353dfa0febf2ac1Test
    https://doi.org/10.1007/s00056-014-0216-xTest


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  10. 10
    Identification of mitochondrial dysfunction in Hutchinson–Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture

    المؤلفون: Pablo Cabezas-Sanchez, Jose L. Luque-Garcia, José Antonio Enríquez, Diego Megias, Carlos López-Otín, Fernando G. Osorio, Vicente Andrés, José Rivera-Torres, Carmen Cámara, Rebeca Acín-Pérez, Cristina González-Gómez

    المساهمون: Ministerio de Economía y Competitividad (España), Instituto de Salud Carlos III, Progeria Research Foundation, Comunidad de Madrid (España), Ministerio de Educación (España), Fundación ProCNIC, Fundación Cajastur

    المصدر: Repisalud
    Instituto de Salud Carlos III (ISCIII)

    مصطلحات موضوعية: Male, Proteomics, Ribosomal protein S6 kinase, 70kDa, polypeptide 1, ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide, ATP5B, Molecular biology of aging, ENO2, Pyruvate kinase, muscle, ATP5F1, Zoledronic Acid, SILAC, Biochemistry, Progerin, LMNA, Mice, Adenosine Triphosphate, Methionine, Progeria, cytochrome c oxidase, Amino Acids, Stable isotope labeling with amino acids in cell culture, Child, p70S6K, Pravastatin, Skin, Mammalian target of rapamycin, Diphosphonates, integumentary system, Imidazoles, ATP5O, Nuclear Proteins, Lamin Type A, MAF, OXPHOS, PKM, Mitochondria, Cell biology, ATP5A1, FTI, mTOR, HGPS, eIF2, Female, eIF4, Flavoprotein subunit of succinate dehydrogenase, CS, Accelerated aging, Premature aging, Senescence, congenital, hereditary, and neonatal diseases and abnormalities, Adolescent, Biophysics, Mouse adult fibroblast, ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide, Biology, ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit, ATP synthase, H+ transporting, mitochondrial F0 complex, subunit B1, Oxygen Consumption, Eukaryotic translation initiation factor 2, Zinc metalloproteinase STE24 homolog, Eukaryotic translation initiation factor 4, ATP5C1, medicine, Animals, Humans, Zmpste24, Oxidative phosphorylation, Protein Precursors, Hutchinson–Gilford progeria syndrome, Enolase 2, COX, Galactose, nutritional and metabolic diseases, Fibroblasts, medicine.disease, Molecular biology, Glucose, ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, Farnesyltransferase inhibitor, Gene Expression Regulation, Mutation, Lamin A, FpSDH, Hydroxymethylglutaryl-CoA Reductase Inhibitors, Mitochondrial dysfunction, citrate synthase

    الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::24117d78cec99c4c6de03d292e120926Test
    https://doi.org/10.1016/j.jprot.2013.08.008Test


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