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  1. Book ; Online: Cellular and Molecular Mechanisms of Nephropathic Cystinosis

    Levtchenko, Elena N.

    2022  

    Keywords Medicine ; Pharmacology ; cystinosis ; cysteamine ; bone ; osteoclast ; genotype ; CD34+ hematopoietic stem and progenitor cells ; gene therapy ; pre-clinical studies ; investigational new drug application ; clinical trial ; disulfiram ; mice ; zebrafish ; fertility ; azoospermia ; hypogonadism ; histopathology ; mouse model ; lysosomal storage disease ; cell and animal models ; infantile nephropathic cystinosis ; bone-muscle wasting ; fibroblast growth factor 23 ; osteoclasts ; sclerostin ; leptin ; fractures ; nephropathic cystinosis ; hollow fiber membrane ; 3-dimensional models ; autophagy ; macrophages ; inflammasome ; proximal tubular cells ; endocytosis ; apoptosis ; chitotriosidase ; interleukins ; galectin-3 ; novel therapies ; endolysosome ; epithelial cell differentiation ; homeostasis ; lysosomal storage diseases ; mitochondrial distress ; kidney proximal tubule ; programmed cell death ; central nervous system ; cortical atrophy ; arterial spin labelling ; cystine blood level ; lysosomal storage disorder ; history ; treatment strategies for cystinosis ; newborn screening ; clinical course ; CTNS-pathogenic variants ; newborn screening for cystinosis ; kidney progenitors ; cell model ; biomarkers ; cystine ; kidney ; therapeutic monitoring
    Language 0|e
    Size 1 electronic resource (230 pages)
    Publisher MDPI - Multidisciplinary Digital Publishing Institute
    Publishing place Basel
    Document type Book ; Online
    Note English ; Open Access
    HBZ-ID HT021613402
    ISBN 9783036545677 ; 3036545670
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Book: Cellular and Molecular Mechanisms of Nephropathic Cystinosis

    Levtchenko, Elena N.

    2022  

    Keywords cysteamine ; Bone
    Language English
    Size 230 p.
    Publisher MDPI AG
    Document type Book
    Note PDA Manuell_16
    Format 175 x 250 x 19
    ISBN 9783036545684 ; 3036545689
    Database PDA

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  3. Book ; Thesis: Cystinosis

    Levtchenko, Elena

    improved medical care and new insights into pathogenesis

    2006  

    Author's details door Elena Nikolaevna Levtchenko
    Language English ; Dutch ; Russian
    Size 180 S. : Ill., graph. Darst.
    Publishing country Netherlands
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Nijmegen, Univ., Diss., 2006
    Note Zsfassung in niederl. und russ. Sprache
    HBZ-ID HT014725077
    ISBN 90-9020326-5 ; 978-90-9020326-3
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2006 E 910 order for loan Magazin

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  4. Article ; Online: A Personal History of Cystinosis by Dr. Jerry Schneider.

    Schneider, Jerry / Levtchenko, Elena

    Cells

    2022  Volume 11, Issue 6

    Abstract: Cystinosis is a rare lysosomal storage disease that is tightly linked with the name of the American physician and scientist Dr. Jerry Schneider. Dr. Schneider (1937-2021) received his medical degree from Northwestern University, followed by a pediatrics ... ...

    Abstract Cystinosis is a rare lysosomal storage disease that is tightly linked with the name of the American physician and scientist Dr. Jerry Schneider. Dr. Schneider (1937-2021) received his medical degree from Northwestern University, followed by a pediatrics residency at Johns Hopkins University and a fellowship in inherited disorders of metabolism. He started to work on cystinosis in J. Seegmiller's laboratory at the National Institutes of Health (NIH) and subsequently moved to the UC San Diego School of Medicine, where he devoted his entire career to people suffering from this devastating lysosomal storage disorder. In 1967, Dr. Schneider's seminal
    MeSH term(s) Child ; Cystinosis/metabolism ; Heterozygote ; History, 21st Century ; Homozygote ; Humans ; Leukocytes/metabolism ; Male ; Medicine ; United States
    Language English
    Publishing date 2022-03-10
    Publishing country Switzerland
    Document type Biography ; Editorial ; Historical Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2661518-6
    ISSN 2073-4409 ; 2073-4409
    ISSN (online) 2073-4409
    ISSN 2073-4409
    DOI 10.3390/cells11060945
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Endocrine Complications of Cystinosis.

    Levtchenko, Elena

    The Journal of pediatrics

    2017  Volume 183S, Page(s) S5–S8

    MeSH term(s) Child ; Child, Preschool ; Cystinosis/complications ; Endocrine Glands/physiopathology ; Endocrine System Diseases/diagnosis ; Endocrine System Diseases/etiology ; Female ; Humans ; Male
    Language English
    Publishing date 2017-03-24
    Publishing country United States
    Document type Journal Article
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2016.12.049
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  6. Article ; Online: Plasma chitotriosidase enzyme activity as a novel therapeutic monitor for cysteamine treatment in nephropathic cystinosis: A retrospective validation study.

    Veys, Koenraad / Elmonem, Mohamed A / van den Heuvel, Lambert / Gahl, William A / Levtchenko, Elena

    Molecular genetics and metabolism

    2024  Volume 142, Issue 1, Page(s) 108454

    Abstract: Background: Cystine-depleting therapy in nephropathic cystinosis is currently monitored via the white blood cell cystine assay, although its application and usefulness are limited by practical and technical issues. Therefore, alternative biomarkers that ...

    Abstract Background: Cystine-depleting therapy in nephropathic cystinosis is currently monitored via the white blood cell cystine assay, although its application and usefulness are limited by practical and technical issues. Therefore, alternative biomarkers that are widely available, more economical and less technically demanding, while reliably reflecting long-term adherence to cysteamine treatment, are desirable. Recently, we proposed chitotriosidase enzyme activity as a potential novel biomarker for the therapeutic monitoring of cysteamine treatment in cystinosis. In this study, we aimed to validate our previous findings and to confirm the value of chitotriosidase in the management of cystinosis therapy.
    Materials & methods: A retrospective study was conducted on 12 patients treated at the National Institutes of Health Clinical Center and followed up for at least 2 years. Plasma chitotriosidase enzyme activity was correlated with corresponding clinical and biochemical data.
    Results: Plasma chitotriosidase enzyme activity significantly correlated with WBC cystine levels, cysteamine total daily dosage and a Composite compliance score. Moreover, plasma chitotriosidase was a significant independent predictor for WBC cystine levels, and cut-off values were established in both non-kidney transplanted and kidney transplanted cystinosis patients to distinguish patients with a good versus poor compliance with cysteamine treatment. Our observations are consistent with those of our previous study and validate our findings.
    Conclusions: Chitotriosidase enzyme activity is a valid potential alternative biomarker for monitoring cysteamine treatment in nephropathic cystinosis patients.
    Synopsis: Chitotriosidase enzyme activity is a valid potential alternative biomarker for monitoring cysteamine treatment in nephropathic cystinosis patients.
    Language English
    Publishing date 2024-03-21
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1418518-0
    ISSN 1096-7206 ; 1096-7192
    ISSN (online) 1096-7206
    ISSN 1096-7192
    DOI 10.1016/j.ymgme.2024.108454
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 617: Show issues Location:
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    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  7. Article ; Online: Residual Cystine Transport Activity for Specific Infantile and Juvenile

    Medaer, Louise / David, Dries / Smits, Maxime / Levtchenko, Elena / Sampaolesi, Maurilio / Gijsbers, Rik

    Cells

    2024  Volume 13, Issue 7

    Abstract: Cystinosis is a rare, autosomal recessive, lysosomal storage disease caused by mutations in the ... ...

    Abstract Cystinosis is a rare, autosomal recessive, lysosomal storage disease caused by mutations in the gene
    MeSH term(s) Humans ; Cystine/metabolism ; Cystinosis/metabolism ; Amino Acid Transport Systems, Neutral/genetics ; Amino Acid Transport Systems, Neutral/metabolism ; Cysteamine ; Mutation/genetics
    Chemical Substances Cystine (48TCX9A1VT) ; Amino Acid Transport Systems, Neutral ; Cysteamine (5UX2SD1KE2) ; CTNS protein, human
    Language English
    Publishing date 2024-04-06
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2661518-6
    ISSN 2073-4409 ; 2073-4409
    ISSN (online) 2073-4409
    ISSN 2073-4409
    DOI 10.3390/cells13070646
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: The potential of RNA-based therapy for kidney diseases.

    Bondue, Tjessa / van den Heuvel, Lambertus / Levtchenko, Elena / Brock, Roland

    Pediatric nephrology (Berlin, Germany)

    2022  

    Abstract: Inherited kidney diseases (IKDs) are a large group of disorders affecting different nephron segments, many of which progress towards kidney failure due to the absence of curative therapies. With the current advances in genetic testing, the understanding ... ...

    Abstract Inherited kidney diseases (IKDs) are a large group of disorders affecting different nephron segments, many of which progress towards kidney failure due to the absence of curative therapies. With the current advances in genetic testing, the understanding of the molecular basis and pathophysiology of these disorders is increasing and reveals new potential therapeutic targets. RNA has revolutionized the world of molecular therapy and RNA-based therapeutics have started to emerge in the kidney field. To apply these therapies for inherited kidney disorders, several aspects require attention. First, the mRNA must be combined with a delivery vehicle that protects the oligonucleotides from degradation in the blood stream. Several types of delivery vehicles have been investigated, including lipid-based, peptide-based, and polymer-based ones. Currently, lipid nanoparticles are the most frequently used formulation for systemic siRNA and mRNA delivery. Second, while the glomerulus and tubules can be reached by charge- and/or size-selectivity, delivery vehicles can also be equipped with antibodies, antibody fragments, targeting peptides, carbohydrates or small molecules to actively target receptors on the proximal tubule epithelial cells, podocytes, mesangial cells or the glomerular endothelium. Furthermore, local injection strategies can circumvent the sequestration of RNA formulations in the liver and physical triggers can also enhance kidney-specific uptake. In this review, we provide an overview of current and potential future RNA-based therapies and targeting strategies that are in development for kidney diseases, with particular interest in inherited kidney disorders.
    Language English
    Publishing date 2022-05-04
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-021-05352-w
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Advances and potential of regenerative medicine in pediatric nephrology.

    Slaats, Gisela G / Chen, Junyu / Levtchenko, Elena / Verhaar, Marianne C / Arcolino, Fanny Oliveira

    Pediatric nephrology (Berlin, Germany)

    2023  Volume 39, Issue 2, Page(s) 383–395

    Abstract: The endogenous capacity of the kidney to repair is limited, and generation of new nephrons after injury for adequate function recovery remains a need. Discovery of factors that promote the endogenous regenerative capacity of the injured kidney or ... ...

    Abstract The endogenous capacity of the kidney to repair is limited, and generation of new nephrons after injury for adequate function recovery remains a need. Discovery of factors that promote the endogenous regenerative capacity of the injured kidney or generation of transplantable kidney tissue represent promising therapeutic strategies. While several encouraging results are obtained after administration of stem or progenitor cells, stem cell secretome, or extracellular vesicles in experimental kidney injury models, very little data exist in the clinical setting to make conclusions about their efficacy. In this review, we provide an overview of the cutting-edge knowledge on kidney regeneration, including pre-clinical methodologies used to elucidate regenerative pathways and describe the perspectives of regenerative medicine for kidney patients.
    MeSH term(s) Child ; Humans ; Kidney ; Regenerative Medicine/methods ; Regeneration ; Nephrology ; Stem Cells/metabolism
    Language English
    Publishing date 2023-07-03
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-023-06039-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 2196: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    ab Jg. 2022: Lesesaal (EG)

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  10. Article ; Online: The Zebrafish Embryo as a Model Organism for Testing mRNA-Based Therapeutics.

    Bondue, Tjessa / Berlingerio, Sante Princiero / van den Heuvel, Lambertus / Levtchenko, Elena

    International journal of molecular sciences

    2023  Volume 24, Issue 13

    Abstract: mRNA-based therapeutics have revolutionized the world of molecular therapy and have proven their potential in the vaccination campaigns for SARS-CoV2 and clinical trials for hereditary disorders. Preclinical studies have mainly focused on in vitro and ... ...

    Abstract mRNA-based therapeutics have revolutionized the world of molecular therapy and have proven their potential in the vaccination campaigns for SARS-CoV2 and clinical trials for hereditary disorders. Preclinical studies have mainly focused on in vitro and rodent studies. However, research in rodents is costly and labour intensive, and requires ethical approval for all interventions. Zebrafish embryonic disease models are not always classified as laboratory animals and have been shown to be extremely valuable for high-throughput drug testing. Zebrafish larvae are characterized by their small size, optical transparency and high number of embryos, and are therefore also suited for the study of mRNA-based therapeutics. First, the one-cell stage injection of naked mRNA can be used to assess the effectivity of gene addition in vivo. Second, the intravascular injection in older larvae can be used to assess tissue targeting efficiency of (packaged) mRNA. In this review, we describe how zebrafish can be used as a steppingstone prior to testing mRNA in rodent models. We define the procedures that can be employed for both the one-cell stage and later-stage injections, as well as the appropriate procedures for post-injection follow-up.
    MeSH term(s) Animals ; Zebrafish/genetics ; RNA, Messenger/genetics ; RNA, Viral ; COVID-19 ; SARS-CoV-2/genetics ; Embryo, Nonmammalian
    Chemical Substances RNA, Messenger ; RNA, Viral
    Language English
    Publishing date 2023-07-07
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms241311224
    Database MEDical Literature Analysis and Retrieval System OnLINE

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