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  1. Article ; Online: Lysosomal cystine accumulation activates mTOR signaling in cystinosis: are mTOR inhibitors the cure?

    Christer, Salómon / Simons, Matias

    Kidney international

    2024  Volume 105, Issue 4, Page(s) 656–658

    MeSH term(s) Humans ; Cystinosis/drug therapy ; Cystine/metabolism ; MTOR Inhibitors ; Lysosomes/metabolism ; TOR Serine-Threonine Kinases ; Amino Acid Transport Systems, Neutral
    Chemical Substances Cystine (48TCX9A1VT) ; MTOR Inhibitors ; TOR Serine-Threonine Kinases (EC 2.7.11.1) ; Amino Acid Transport Systems, Neutral
    Language English
    Publishing date 2024-02-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2023.11.030
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: ER stress and slit diaphragms: is there a connection?

    Hermle, Tobias / Simons, Matias

    Kidney international

    2023  Volume 103, Issue 5, Page(s) 830–832

    Abstract: Galloway-Mowat syndrome is a neurorenal syndrome that has been linked with defective transfer RNA and protein translation caused by variants in the multiprotein complex KEOPS. In the kidney, this syndrome seems to primarily affect the podocytes, but the ... ...

    Abstract Galloway-Mowat syndrome is a neurorenal syndrome that has been linked with defective transfer RNA and protein translation caused by variants in the multiprotein complex KEOPS. In the kidney, this syndrome seems to primarily affect the podocytes, but the pathogenesis has remained unclear. In this issue of Kidney International, Krausel et al. use Drosophila nephrocytes to link endoplasmic reticulum stress with defects in the slit diaphragm.
    MeSH term(s) Animals ; Membrane Proteins/genetics ; Drosophila/metabolism ; Podocytes/metabolism ; Nephrosis/metabolism ; Drosophila Proteins/genetics ; Drosophila Proteins/metabolism
    Chemical Substances Membrane Proteins ; Drosophila Proteins
    Language English
    Publishing date 2023-04-21
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Comment
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2023.01.028
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Book ; Thesis: Zelluläre Mechanismen im Kontext der glomerulären Filtration

    Simons, Matias

    molekulare Charakterisierung der Fortsatzbildung von Podozyten und des Aufbaus der glomerulären Schlitzmembran

    2002  

    Author's details vorgelegt von Matias Simons
    Language German
    Size 109 Bl., Ill., graph. Darst., 30 cm
    Publishing country Germany
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Heidelberg, Univ., Diss., 2002
    HBZ-ID HT013514430
    Database Catalogue ZB MED Medicine, Health

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  4. Article ; Online: The Benefits of Tubular Proteinuria: An Evolutionary Perspective.

    Simons, Matias

    Journal of the American Society of Nephrology : JASN

    2018  Volume 29, Issue 3, Page(s) 710–712

    MeSH term(s) Albumins/metabolism ; Animals ; Evolution, Molecular ; Humans ; Kidney Tubules, Proximal/physiopathology ; Low Density Lipoprotein Receptor-Related Protein-2/genetics ; Low Density Lipoprotein Receptor-Related Protein-2/metabolism ; Membrane Proteins ; Mice ; Proteins/genetics ; Proteins/metabolism ; Proteinuria/genetics ; Proteinuria/physiopathology ; Receptors, Cell Surface/genetics ; Receptors, Cell Surface/metabolism ; Renal Reabsorption
    Chemical Substances AMN protein, human ; Albumins ; Low Density Lipoprotein Receptor-Related Protein-2 ; Membrane Proteins ; Proteins ; Receptors, Cell Surface ; intrinsic factor-cobalamin receptor
    Language English
    Publishing date 2018-01-25
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1085942-1
    ISSN 1533-3450 ; 1046-6673
    ISSN (online) 1533-3450
    ISSN 1046-6673
    DOI 10.1681/ASN.2017111197
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Publisher Correction: Drosophila melanogaster: a simple genetic model of kidney structure, function and disease.

    Dow, Julian A T / Simons, Matias / Romero, Michael F

    Nature reviews. Nephrology

    2023  Volume 20, Issue 1, Page(s) 70

    Language English
    Publishing date 2023-11-10
    Publishing country England
    Document type Published Erratum
    ZDB-ID 2490366-8
    ISSN 1759-507X ; 1759-5061
    ISSN (online) 1759-507X
    ISSN 1759-5061
    DOI 10.1038/s41581-023-00788-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Drosophila melanogaster: a simple genetic model of kidney structure, function and disease.

    Dow, Julian A T / Simons, Matias / Romero, Michael F

    Nature reviews. Nephrology

    2022  Volume 18, Issue 7, Page(s) 417–434

    Abstract: Although the genetic basis of many kidney diseases is being rapidly elucidated, their experimental study remains problematic owing to the lack of suitable models. The fruitfly Drosophila melanogaster provides a rapid, ethical and cost-effective model ... ...

    Abstract Although the genetic basis of many kidney diseases is being rapidly elucidated, their experimental study remains problematic owing to the lack of suitable models. The fruitfly Drosophila melanogaster provides a rapid, ethical and cost-effective model system of the kidney. The unique advantages of D. melanogaster include ease and low cost of maintenance, comprehensive availability of genetic mutants and powerful transgenic technologies, and less onerous regulation, as compared with mammalian systems. Renal and excretory functions in D. melanogaster reside in three main tissues - the transporting renal (Malpighian) tubules, the reabsorptive hindgut and the endocytic nephrocytes. Tubules contain multiple cell types and regions and generate a primary urine by transcellular transport rather than filtration, which is then subjected to selective reabsorption in the hindgut. By contrast, the nephrocytes are specialized for uptake of macromolecules and equipped with a filtering slit diaphragm resembling that of podocytes. Many genes with key roles in the human kidney have D. melanogaster orthologues that are enriched and functionally relevant in fly renal tissues. This similarity has allowed investigations of epithelial transport, kidney stone formation and podocyte and proximal tubule function. Furthermore, a range of unique quantitative phenotypes are available to measure function in both wild type and disease-modelling flies.
    MeSH term(s) Animals ; Drosophila Proteins/genetics ; Drosophila Proteins/metabolism ; Drosophila melanogaster/genetics ; Drosophila melanogaster/metabolism ; Humans ; Kidney/metabolism ; Kidney Calculi ; Mammals/metabolism ; Models, Genetic
    Chemical Substances Drosophila Proteins
    Language English
    Publishing date 2022-04-11
    Publishing country England
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
    ZDB-ID 2490366-8
    ISSN 1759-507X ; 1759-5061
    ISSN (online) 1759-507X
    ISSN 1759-5061
    DOI 10.1038/s41581-022-00561-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Flies With Skin Blisters.

    Simons, Matias

    The Journal of investigative dermatology

    2015  Volume 135, Issue 8, Page(s) 1944–1945

    Abstract: Epidermolysis bullosa simplex (EBS) belongs to a family of genetic conditions that cause the skin to be fragile and to blister easily. Although most of the genes involved are known, the molecular mechanisms underlying keratin aggregation remain obscure. ... ...

    Abstract Epidermolysis bullosa simplex (EBS) belongs to a family of genetic conditions that cause the skin to be fragile and to blister easily. Although most of the genes involved are known, the molecular mechanisms underlying keratin aggregation remain obscure. In this issue of the Journal, Bohnekamp et al. report on a novel EBS model that is based on the de novo formation of keratin filaments in "keratin null flies."
    MeSH term(s) Animals ; Disease Models, Animal ; Drosophila/metabolism ; Epidermolysis Bullosa Simplex/metabolism ; Epidermolysis Bullosa Simplex/pathology ; Humans
    Language English
    Publishing date 2015-08
    Publishing country United States
    Document type Comment ; Journal Article
    ZDB-ID 80136-7
    ISSN 1523-1747 ; 0022-202X
    ISSN (online) 1523-1747
    ISSN 0022-202X
    DOI 10.1038/jid.2015.193
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article: Model organisms for functional validation in genetic renal disease

    Boettcher, Susanne / Simons, Matias

    Medizinische Genetik

    2022  Volume 34, Issue 4, Page(s) 287

    Language German
    Document type Article
    ZDB-ID 1083376-6
    ISSN 0936-5931
    Database Current Contents Medicine

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  9. Article ; Online: Filling the Gap:

    Marelja, Zvonimir / Simons, Matias

    Journal of the American Society of Nephrology : JASN

    2019  Volume 30, Issue 5, Page(s) 719–720

    MeSH term(s) Animals ; Drosophila ; Drosophila Proteins/genetics ; Kidney ; Mutation ; Nephrotic Syndrome/genetics
    Chemical Substances Drosophila Proteins
    Language English
    Publishing date 2019-03-25
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 1085942-1
    ISSN 1533-3450 ; 1046-6673
    ISSN (online) 1533-3450
    ISSN 1046-6673
    DOI 10.1681/ASN.2019020181
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: The (pro)renin receptor: what's in a name?

    Simons, Matias / Bader, Michael / Müller, Dominik N

    Nature reviews. Nephrology

    2020  Volume 16, Issue 5, Page(s) 304

    MeSH term(s) Renin
    Chemical Substances Renin (EC 3.4.23.15)
    Language English
    Publishing date 2020-05-12
    Publishing country England
    Document type Letter ; Comment
    ZDB-ID 2490366-8
    ISSN 1759-507X ; 1759-5061
    ISSN (online) 1759-507X
    ISSN 1759-5061
    DOI 10.1038/s41581-020-0274-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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