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  1. Article ; Online: Energy metabolism disorders in rare and common diseases. Toward bioenergetic modulation therapy and the training of a new generation of European scientists.

    Rossignol, Rodrigue

    The international journal of biochemistry & cell biology

    2015  Volume 63, Page(s) 2–9

    Abstract: Energy metabolism alterations are found in a large number of rare and common diseases of genetic or environmental origin. The number of patients that could benefit from bioenergetic modulation therapy (BIOMET) is therefore very important and includes ... ...

    Abstract Energy metabolism alterations are found in a large number of rare and common diseases of genetic or environmental origin. The number of patients that could benefit from bioenergetic modulation therapy (BIOMET) is therefore very important and includes individuals with pathologies as diverse as mitochondrial diseases, acute coronary syndrome, chronic kidney disease, asthma or even cancer. Although, the alteration of energy metabolism is disease specific and sometimes patient specific, the strategies for BIOMET could be common and target a series of bioenergetic regulatory mechanisms discussed in this article. An excellent training of scientists in the field of energy metabolism, related human diseases and drug discovery is also crucial to form a young generation of MDs, PHDs and Pharma or CRO-group leaders who will discover novel personalized bioenergetic medicines, through pharmacology, genetics, nutrition or adapted exercise training. The Mitochondrial European Educational Training (MEET) consortium was created to pursue this goal, and we dedicated here a special issue of Organelle in Focus (OiF) to highlight their objectives. A total of 10 OiFs articles constitute this Directed Issue on Mitochondrial Medicine. As part of this editorial article, we asked timely questions to the PR. Jan W. Smeitink, professor of Mitochondrial Medicine and CEO of Khondrion, a mitochondrial medicine company. He shared with us his objectives and strategies for the study of mitochondrial diseases and the identification of future treatments. This article is part of a Directed Issue entitled: Energy Metabolism Disorders and Therapies.
    MeSH term(s) Energy Metabolism ; Europe ; Humans ; Metabolic Diseases/metabolism ; Metabolic Diseases/therapy ; Mitochondrial Diseases/metabolism ; Mitochondrial Diseases/therapy ; Research
    Language English
    Publishing date 2015-06
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1228429-4
    ISSN 1878-5875 ; 1357-2725
    ISSN (online) 1878-5875
    ISSN 1357-2725
    DOI 10.1016/j.biocel.2015.01.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: The deubiquitinase OTUB1 governs lung cancer cell fitness by modulating proteostasis of OXPHOS proteins.

    Sheryazdanova, Aidana / Amoedo, Nivea Dias / Dufour, Sara / Impens, Francis / Rossignol, Rodrigue / Sablina, Anna

    Biochimica et biophysica acta. Molecular basis of disease

    2023  Volume 1869, Issue 7, Page(s) 166767

    Abstract: Aerobic glycolysis is a hallmark of cancer development, but this dogma has been challenged by reports showing a key role of oxidative phosphorylation (OXPHOS) in cancer cell survival. It has been proposed that increased levels of intramitochondrial ... ...

    Abstract Aerobic glycolysis is a hallmark of cancer development, but this dogma has been challenged by reports showing a key role of oxidative phosphorylation (OXPHOS) in cancer cell survival. It has been proposed that increased levels of intramitochondrial proteins in cancer cells are associated with high OXPHOS activity and increased sensitivity to OXPHOS inhibitors. However, the molecular mechanisms leading to the high expression of OXPHOS proteins in cancer cells remain unknown. Multiple proteomics studies have detected the ubiquitination of intramitochondrial proteins, suggesting the contribution of the ubiquitin system to the proteostatic regulation of OXPHOS proteins. Here, we identified the ubiquitin hydrolase OTUB1 as a regulator of the mitochondrial metabolic machinery essential for lung cancer cell survival. Mitochondria-localized OTUB1 modulates respiration by inhibiting K48-linked ubiquitination and turnover of OXPHOS proteins. An increase in OTUB1 expression is commonly observed in one-third of non-small-cell lung carcinomas and is associated with high OXPHOS signatures. Moreover, OTUB1 expression highly correlates with the sensitivity of lung cancer cells to mitochondrial inhibitors.
    MeSH term(s) Humans ; Oxidative Phosphorylation ; Lung Neoplasms/genetics ; Carcinoma, Non-Small-Cell Lung/genetics ; Proteostasis ; Ubiquitin/metabolism ; Deubiquitinating Enzymes/metabolism
    Chemical Substances Ubiquitin ; Deubiquitinating Enzymes (EC 3.4.19.12)
    Language English
    Publishing date 2023-05-26
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 60-7
    ISSN 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 ; 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    ISSN (online) 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650
    ISSN 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    DOI 10.1016/j.bbadis.2023.166767
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Bioenergetics of cancer. Foreword.

    Rossignol, Rodrigue

    Biochimica et biophysica acta

    2011  Volume 1807, Issue 6, Page(s) 533

    MeSH term(s) Antineoplastic Agents/chemical synthesis ; Antineoplastic Agents/therapeutic use ; Energy Metabolism/physiology ; Humans ; Metabolic Networks and Pathways/genetics ; Mitochondria/metabolism ; Mitochondria/physiology ; Molecular Targeted Therapy/methods ; Neoplasms/genetics ; Neoplasms/metabolism ; Neoplasms/physiopathology ; Precision Medicine/methods ; Precision Medicine/trends
    Chemical Substances Antineoplastic Agents
    Language English
    Publishing date 2011-06
    Publishing country Netherlands
    Document type Editorial ; Introductory Journal Article
    ZDB-ID 60-7
    ISSN 1879-2596 ; 1879-260X ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 ; 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    ISSN (online) 1879-2596 ; 1879-260X ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650
    ISSN 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    DOI 10.1016/j.bbabio.2011.03.005
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Energy metabolism disorders in rare and common diseases. Toward bioenergetic modulation therapy and the training of a new generation of European scientists

    Rossignol, Rodrigue

    international journal of biochemistry & cell biology. 2015 June, v. 63

    2015  

    Abstract: Energy metabolism alterations are found in a large number of rare and common diseases of genetic or environmental origin. The number of patients that could benefit from bioenergetic modulation therapy (BIOMET) is therefore very important and includes ... ...

    Abstract Energy metabolism alterations are found in a large number of rare and common diseases of genetic or environmental origin. The number of patients that could benefit from bioenergetic modulation therapy (BIOMET) is therefore very important and includes individuals with pathologies as diverse as mitochondrial diseases, acute coronary syndrome, chronic kidney disease, asthma or even cancer. Although, the alteration of energy metabolism is disease specific and sometimes patient specific, the strategies for BIOMET could be common and target a series of bioenergetic regulatory mechanisms discussed in this article. An excellent training of scientists in the field of energy metabolism, related human diseases and drug discovery is also crucial to form a young generation of MDs, PHDs and Pharma or CRO-group leaders who will discover novel personalized bioenergetic medicines, through pharmacology, genetics, nutrition or adapted exercise training. The Mitochondrial European Educational Training (MEET) consortium was created to pursue this goal, and we dedicated here a special issue of Organelle in Focus (OiF) to highlight their objectives. A total of 10 OiFs articles constitute this Directed Issue on Mitochondrial Medicine. As part of this editorial article, we asked timely questions to the PR. Jan W. Smeitink, professor of Mitochondrial Medicine and CEO of Khondrion, a mitochondrial medicine company. He shared with us his objectives and strategies for the study of mitochondrial diseases and the identification of future treatments.This article is part of a Directed Issue entitled: Energy Metabolism Disorders and Therapies.
    Keywords asthma ; drugs ; energy metabolism ; exercise ; genetics ; human diseases ; kidney diseases ; medicine ; mitochondria ; neoplasms ; nutrition ; patients ; pharmacology ; scientists ; therapeutics ; training (people)
    Language English
    Dates of publication 2015-06
    Size p. 2-9.
    Publishing place Elsevier Ltd
    Document type Article
    ZDB-ID 1228429-4
    ISSN 1878-5875 ; 1357-2725
    ISSN (online) 1878-5875
    ISSN 1357-2725
    DOI 10.1016/j.biocel.2015.01.003
    Database NAL-Catalogue (AGRICOLA)

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  5. Article ; Online: Proteomic Study of Low-Birth-Weight Nephropathy in Rats.

    Imasawa, Toshiyuki / Claverol, Stéphane / Lacombe, Didier / Amoedo, Nivea Dias / Rossignol, Rodrigue

    International journal of molecular sciences

    2021  Volume 22, Issue 19

    Abstract: The hyperfiltration theory has been used to explain the mechanism of low birth weight (LBW)-related nephropathy. However, the molecular changes in the kidney proteome have not been defined in this disease, and early biomarkers are lacking. We ... ...

    Abstract The hyperfiltration theory has been used to explain the mechanism of low birth weight (LBW)-related nephropathy. However, the molecular changes in the kidney proteome have not been defined in this disease, and early biomarkers are lacking. We investigated the molecular pathogenesis of LBW rats obtained by intraperitoneal injection of dexamethasone into pregnant animals. Normal-birth-weight (NBW) rats were used as controls. When the rats were four weeks old, the left kidneys were removed and used for comprehensive label-free proteomic studies. Following uninephrectomy, all rats were fed a high-salt diet until 9 weeks of age. Differences in the molecular composition of the kidney cortex were observed at the early step of LBW nephropathy pathogenesis. Untargeted quantitative proteomics showed that proteins involved in energy metabolism, such as oxidative phosphorylation (OXPHOS), the TCA cycle, and glycolysis, were specifically downregulated in the kidneys of LBW rats at four weeks. No pathological changes were detected at this early stage. Pathway analysis identified NEFL2 (NRF2) and RICTOR as potential upstream regulators. The search for biomarkers identified components of the mitochondrial respiratory chain, namely, ubiquinol-cytochrome c reductase complex subunits (UQCR7/11) and ATP5I/L, two components of mitochondrial F
    MeSH term(s) Animals ; Female ; Male ; Pregnancy ; Rats ; Animals, Newborn/metabolism ; Biomarkers/metabolism ; Birth Weight/physiology ; Electron Transport Complex III/metabolism ; Kidney/metabolism ; Kidney Diseases/metabolism ; NF-E2-Related Factor 2/metabolism ; Oxidative Phosphorylation ; Proteome/metabolism ; Proteomics/methods ; Rapamycin-Insensitive Companion of mTOR Protein/metabolism
    Chemical Substances Biomarkers ; Electron Transport Complex III (EC 7.1.1.8) ; NF-E2-Related Factor 2 ; Proteome ; Rapamycin-Insensitive Companion of mTOR Protein
    Language English
    Publishing date 2021-09-24
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms221910294
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Mutation on MT-CO2 gene induces mitochondrial disease associated with neurodegeneration and intracerebral iron accumulation (NBIA).

    Courtois, Sarah / Angelini, Chloé / M Durand, Christelle / Dias Amoedo, Nivea / Courreges, Armelle / Dumon, Elodie / Le Quang, Mégane / Goizet, Cyril / Martin-Negrier, Marie-Laure / Rossignol, Rodrigue / Lacombe, Didier / Coupry, Isabelle / Trimouille, Aurélien

    Biochimica et biophysica acta. Molecular basis of disease

    2023  Volume 1870, Issue 1, Page(s) 166856

    Abstract: Mitochondrial diseases are genetic disorders impairing mitochondrial functions. Here we describe a patient with a neurodegenerative condition associated with myopia, bilateral sensorineural hearing loss and motor disorders. Brain MRIs showed major ... ...

    Abstract Mitochondrial diseases are genetic disorders impairing mitochondrial functions. Here we describe a patient with a neurodegenerative condition associated with myopia, bilateral sensorineural hearing loss and motor disorders. Brain MRIs showed major cortico-subcortical and infra-tentorial atrophies, as well as intracerebral iron accumulation and central calcifications, compatible with a NBIA-like phenotype. Mitochondrial DNA analysis revealed an undescribed variant: m.8091G>A in the MT-CO2 gene, associated with a complex IV deficiency and a decrease of the mitochondrial respiratory chain capabilities. We report here this pathogenic variant, associated with a NBIA-like phenotype.
    MeSH term(s) Humans ; Carbon Dioxide ; Mutation ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/pathology ; Mitochondrial Diseases/complications ; Iron
    Chemical Substances Carbon Dioxide (142M471B3J) ; Iron (E1UOL152H7)
    Language English
    Publishing date 2023-08-26
    Publishing country Netherlands
    Document type Case Reports ; Journal Article
    ZDB-ID 60-7
    ISSN 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 ; 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    ISSN (online) 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650
    ISSN 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    DOI 10.1016/j.bbadis.2023.166856
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: A Mechanism Underpinning the Bioenergetic Metabolism-Regulating Function of Gold Nanocatalysts.

    Wang, Zixin / Henriques, Alexandre / Rouvière, Laura / Callizot, Noëlle / Tan, Lin / Hotchkin, Michael T / Rossignol, Rodrigue / Mortenson, Mark G / Dorfman, Adam R / Ho, Karen S / Wang, Hui

    Small (Weinheim an der Bergstrasse, Germany)

    2023  Volume 20, Issue 8, Page(s) e2304082

    Abstract: Bioenergetic deficits are known to be significant contributors to neurodegenerative diseases. Nevertheless, identifying safe and effective means to address intracellular bioenergetic deficits remains a significant challenge. This work provides ... ...

    Abstract Bioenergetic deficits are known to be significant contributors to neurodegenerative diseases. Nevertheless, identifying safe and effective means to address intracellular bioenergetic deficits remains a significant challenge. This work provides mechanistic insights into the energy metabolism-regulating function of colloidal Au nanocrystals, referred to as CNM-Au8, that are synthesized electrochemically in the absence of surface-capping organic ligands. When neurons are subjected to excitotoxic stressors or toxic peptides, treatment of neurons with CNM-Au8 results in dose-dependent neuronal survival and neurite network preservation across multiple neuronal subtypes. CNM-Au8 efficiently catalyzes the conversion of an energetic cofactor, nicotinamide adenine dinucleotide hydride (NADH), into its oxidized counterpart (NAD
    MeSH term(s) Humans ; NAD/chemistry ; Gold/chemistry ; Oxidation-Reduction ; Neurodegenerative Diseases
    Chemical Substances NAD (0U46U6E8UK) ; Gold (7440-57-5)
    Language English
    Publishing date 2023-09-28
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 2168935-0
    ISSN 1613-6829 ; 1613-6810
    ISSN (online) 1613-6829
    ISSN 1613-6810
    DOI 10.1002/smll.202304082
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Proteomic Study of Low-Birth-Weight Nephropathy in Rats

    Toshiyuki Imasawa / Stéphane Claverol / Didier Lacombe / Nivea Dias Amoedo / Rodrigue Rossignol

    International Journal of Molecular Sciences, Vol 22, Iss 10294, p

    2021  Volume 10294

    Abstract: The hyperfiltration theory has been used to explain the mechanism of low birth weight (LBW)-related nephropathy. However, the molecular changes in the kidney proteome have not been defined in this disease, and early biomarkers are lacking. We ... ...

    Abstract The hyperfiltration theory has been used to explain the mechanism of low birth weight (LBW)-related nephropathy. However, the molecular changes in the kidney proteome have not been defined in this disease, and early biomarkers are lacking. We investigated the molecular pathogenesis of LBW rats obtained by intraperitoneal injection of dexamethasone into pregnant animals. Normal-birth-weight (NBW) rats were used as controls. When the rats were four weeks old, the left kidneys were removed and used for comprehensive label-free proteomic studies. Following uninephrectomy, all rats were fed a high-salt diet until 9 weeks of age. Differences in the molecular composition of the kidney cortex were observed at the early step of LBW nephropathy pathogenesis. Untargeted quantitative proteomics showed that proteins involved in energy metabolism, such as oxidative phosphorylation (OXPHOS), the TCA cycle, and glycolysis, were specifically downregulated in the kidneys of LBW rats at four weeks. No pathological changes were detected at this early stage. Pathway analysis identified NEFL2 (NRF2) and RICTOR as potential upstream regulators. The search for biomarkers identified components of the mitochondrial respiratory chain, namely, ubiquinol-cytochrome c reductase complex subunits (UQCR7/11) and ATP5I/L, two components of mitochondrial F 1 F O -ATP synthase. These findings were further validated by immunohistology. At later stages of the disease process, the right kidneys revealed an increased frequency of focal segmental glomerulosclerosis lesions, interstitial fibrosis and tubular atrophy. Our findings revealed proteome changes in LBW rat kidneys and revealed a strong downregulation of specific mitochondrial respiratory chain proteins, such as UQCR7.
    Keywords low birth weight ; nephropathy ; proteomics ; kidney ; mitochondria ; Biology (General) ; QH301-705.5 ; Chemistry ; QD1-999
    Subject code 630
    Language English
    Publishing date 2021-09-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article ; Online: Metabolic reprogramming by tobacco-specific nitrosamines (TSNAs) in cancer.

    Sarlak, Saharnaz / Lalou, Claude / Amoedo, Nivea Dias / Rossignol, Rodrigue

    Seminars in cell & developmental biology

    2019  Volume 98, Page(s) 154–166

    Abstract: Metabolic reprogramming is a hallmark of cancer and the link between oncogenes activation, tumor supressors inactivation and bioenergetics modulation is well established. However, numerous carcinogenic environmental factors are responsible for early ... ...

    Abstract Metabolic reprogramming is a hallmark of cancer and the link between oncogenes activation, tumor supressors inactivation and bioenergetics modulation is well established. However, numerous carcinogenic environmental factors are responsible for early cancer initiation and their impact on metabolic reprogramming just starts to be deciphered. For instance, it was recently shown that UVB irradiation triggers metabolic reprogramming at the pre-cancer stage with implication for skin cancer detection and therapy. These observations foster the need to study the early changes in tissue metabolism following exposure to other carcinogenic events. According to the International Agency for Research on Cancer (IARC), tobacco smoke is a major class I-carcinogenic environmental factor that contains different carcinogens, but little is known on the impact of tobacco smoke on tissue metabolism and its participation to cancer initiation. In particular, tobacco-specific nitrosamines (TSNAs) play a central role in tobacco-smoke mediated cancer initiation. Here we describe the recent advances that have led to a new hypothesis regarding the link between nitrosamines signaling and metabolic reprogramming in cancer.
    MeSH term(s) Cellular Reprogramming ; Humans ; Neoplasms/metabolism ; Neoplasms/pathology ; Nitrosamines/metabolism ; Nicotiana/chemistry
    Chemical Substances Nitrosamines
    Language English
    Publishing date 2019-11-04
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1312473-0
    ISSN 1096-3634 ; 1084-9521
    ISSN (online) 1096-3634
    ISSN 1084-9521
    DOI 10.1016/j.semcdb.2019.09.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: Mitochondria in cancer.

    Gasparre, Giuseppe / Rossignol, Rodrigue / Sonveaux, Pierre

    Biochimica et biophysica acta. Bioenergetics

    2017  Volume 1858, Issue 8, Page(s) 553–555

    MeSH term(s) Animals ; Biological Transport ; Energy Metabolism ; Humans ; Membrane Transport Proteins/metabolism ; Mitochondria/metabolism ; Mitochondrial Membranes/metabolism ; Mitochondrial Proteins/metabolism ; Neoplasms/metabolism ; Neoplasms/therapy
    Chemical Substances Membrane Transport Proteins ; Mitochondrial Proteins
    Language English
    Publishing date 2017-06-26
    Publishing country Netherlands
    Document type Introductory Journal Article
    ZDB-ID 60-7
    ISSN 1879-2596 ; 1879-260X ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 ; 0005-2728 ; 0006-3002 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    ISSN (online) 1879-2596 ; 1879-260X ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650
    ISSN 0005-2728 ; 0006-3002 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399
    DOI 10.1016/j.bbabio.2017.05.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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