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  1. Article: Editorial: Women in renal pharmacology: 2021.

    Benigni, Ariela / Tomasoni, Susanna

    Frontiers in pharmacology

    2022  Volume 13, Page(s) 1054354

    Language English
    Publishing date 2022-10-21
    Publishing country Switzerland
    Document type Editorial
    ZDB-ID 2587355-6
    ISSN 1663-9812
    ISSN 1663-9812
    DOI 10.3389/fphar.2022.1054354
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Imaging the Kidney with an Unconventional Scanning Electron Microscopy Technique: Analysis of the Subpodocyte Space in Diabetic Mice.

    Conti, Sara / Remuzzi, Giuseppe / Benigni, Ariela / Tomasoni, Susanna

    International journal of molecular sciences

    2022  Volume 23, Issue 3

    Abstract: Transmission electron microscopy (TEM) remains the gold standard for renal histopathological diagnoses, given its higher resolving power, compared with light microscopy. However, it imposes several limitations on pathologists, including longer sample ... ...

    Abstract Transmission electron microscopy (TEM) remains the gold standard for renal histopathological diagnoses, given its higher resolving power, compared with light microscopy. However, it imposes several limitations on pathologists, including longer sample preparation time and a small observation area. To overcome these, we introduced a scanning electron microscopy (SEM) technique for imaging resin-embedded semi-thin sections of renal tissue. We developed a rapid tissue preparation protocol for experimental models and human biopsies which, alongside SEM digital imaging acquisition of secondary electrons (SE-SEM), enables fast electron microscopy examination, with a resolution similar to that achieved by TEM. We used this unconventional SEM imaging approach to investigate the subpodocyte space (SPS) in BTBR
    MeSH term(s) Animals ; Diabetes Mellitus, Experimental/pathology ; Diabetes Mellitus, Type 2/pathology ; Glomerular Filtration Barrier/ultrastructure ; Mice ; Microscopy, Electron, Scanning ; Podocytes/ultrastructure
    Language English
    Publishing date 2022-02-01
    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/ijms23031699
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Generation of a homozygous CIITA knockout iPS cell line using the CRISPR-Cas9 system.

    Romano, Elena / Trionfini, Piera / Giampietro, Roberta / Benigni, Ariela / Tomasoni, Susanna

    Stem cell research

    2021  Volume 57, Page(s) 102580

    Abstract: Human induced pluripotent stem cells (iPSCs) have great promise in regenerative medicine. However, several limitations, including immune-incompatibility, have raised concerns regarding their clinical application. Recent studies have shown that human ... ...

    Abstract Human induced pluripotent stem cells (iPSCs) have great promise in regenerative medicine. However, several limitations, including immune-incompatibility, have raised concerns regarding their clinical application. Recent studies have shown that human iPSCs and their derivatives lose their immunogenicity when major histocompatibility complex (MHC) class I and II genes are inactivated and CD47 is over-expressed. In this study, we used CRISPR-Cas9 technology to generate an isogenic iPSC line with a homozygous frameshift mutation in the MHC II transactivator (CIITA) gene. The CIITA
    Language English
    Publishing date 2021-10-18
    Publishing country England
    Document type Journal Article
    ZDB-ID 2393143-7
    ISSN 1876-7753 ; 1873-5061
    ISSN (online) 1876-7753
    ISSN 1873-5061
    DOI 10.1016/j.scr.2021.102580
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  4. Article ; Online: Long-term adaptive response in COVID-19 vaccine recipients and the effect of a booster dose.

    Perico, Luca / Todeschini, Marta / Casiraghi, Federica / Mister, Marilena / Pezzotta, Anna / Peracchi, Tobia / Tomasoni, Susanna / Trionfini, Piera / Benigni, Ariela / Remuzzi, Giuseppe

    Frontiers in immunology

    2023  Volume 14, Page(s) 1123158

    Abstract: We examined the immune response in subjects previously infected with SARS-CoV2 and infection-naïve 9 months after primary 2-dose COVID-19 mRNA vaccination and 3 months after the booster dose in a longitudinal cohort of healthcare workers. Nine months ... ...

    Abstract We examined the immune response in subjects previously infected with SARS-CoV2 and infection-naïve 9 months after primary 2-dose COVID-19 mRNA vaccination and 3 months after the booster dose in a longitudinal cohort of healthcare workers. Nine months after primary vaccination, previously infected subjects exhibited higher residual antibody levels, with significant neutralizing activity against distinct variants compared to infection-naïve subjects. The higher humoral response was associated with higher levels of receptor binding domain (RBD)-specific IgG
    MeSH term(s) Humans ; COVID-19 Vaccines ; COVID-19/prevention & control ; RNA, Viral ; SARS-CoV-2 ; Antibodies, Neutralizing
    Chemical Substances COVID-19 Vaccines ; RNA, Viral ; Antibodies, Neutralizing
    Language English
    Publishing date 2023-02-28
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2023.1123158
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  5. Article ; Online: Efficacy of GalNAc C3 siRNAs in factor H-deficient mice with C3 glomerulopathy.

    Zanchi, Cristina / Locatelli, Monica / Cerullo, Domenico / Aumiller, Verena / Corna, Daniela / Rottoli, Daniela / Schubert, Steffen / Noris, Marina / Tomasoni, Susanna / Remuzzi, Giuseppe / Zoja, Carlamaria / Benigni, Ariela

    Molecular immunology

    2024  Volume 168, Page(s) 10–16

    Abstract: Complement alternative pathway (AP) dysregulation drives C3 glomerulopathy (C3G), a rare renal disorder characterized by glomerular C3 deposition and glomerular damage, for which no effective treatments are available. Blockade of complement C3 is ... ...

    Abstract Complement alternative pathway (AP) dysregulation drives C3 glomerulopathy (C3G), a rare renal disorder characterized by glomerular C3 deposition and glomerular damage, for which no effective treatments are available. Blockade of complement C3 is emerging as a viable therapeutic option. In an earlier study we showed that SLN500, a small interfering RNA targeting liver C3 synthesis, was able to limit AP dysregulation and glomerular C3d deposits in mice with partial factor H (FH) deficiency (Cfh
    MeSH term(s) Humans ; Animals ; Mice ; Complement C3/genetics ; Complement C3/metabolism ; RNA, Small Interfering/genetics ; RNA, Small Interfering/therapeutic use ; Complement Factor H/genetics ; Complement Factor H/therapeutic use ; Complement Factor H/deficiency ; Glomerulonephritis, Membranoproliferative/genetics ; Glomerulonephritis, Membranoproliferative/drug therapy ; Glomerulonephritis, Membranoproliferative/metabolism ; Kidney Diseases ; Complement Pathway, Alternative ; Hereditary Complement Deficiency Diseases
    Chemical Substances Complement C3 ; RNA, Small Interfering ; Complement Factor H (80295-65-4)
    Language English
    Publishing date 2024-02-17
    Publishing country England
    Document type Journal Article
    ZDB-ID 424427-8
    ISSN 1872-9142 ; 0161-5890
    ISSN (online) 1872-9142
    ISSN 0161-5890
    DOI 10.1016/j.molimm.2024.02.010
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 166: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  6. Article ; Online: Generation of PKD1 mono-allelic and bi-allelic knockout iPS cell lines using CRISPR-Cas9 system.

    Romano, Elena / Trionfini, Piera / Ciampi, Osele / Benigni, Ariela / Tomasoni, Susanna

    Stem cell research

    2020  Volume 47, Page(s) 101881

    Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, characterised by the development of multiple fluid-filled cysts in the kidneys and other organs. PKD1 and PKD2 are the two major causative genes encoding ... ...

    Abstract Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, characterised by the development of multiple fluid-filled cysts in the kidneys and other organs. PKD1 and PKD2 are the two major causative genes encoding for polycystin-1 and polycystin-2, respectively. Here, we report the generation of two isogenic induced pluripotent stem cell (iPSC) lines with either heterozygous or compound heterozygous mutations in the PKD1 gene using CRISPR-Cas9 technology. The PKD1
    Language English
    Publishing date 2020-06-19
    Publishing country England
    Document type Journal Article
    ZDB-ID 2393143-7
    ISSN 1876-7753 ; 1873-5061
    ISSN (online) 1876-7753
    ISSN 1873-5061
    DOI 10.1016/j.scr.2020.101881
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  7. Article ; Online: Generation of two isogenic iPS cell lines (IRFMNi002-A and IRFMNi002-B) from a patient affected by Focal Segmental Glomerulosclerosis carrying a heterozygous c.565G>A mutation in PAX2 gene.

    Ciampi, Osele / Romano, Elena / Benigni, Ariela / Tomasoni, Susanna

    Stem cell research

    2018  Volume 33, Page(s) 175–179

    Abstract: Focal Segmental Glomerulosclerosis (FSGS) is the typical renal histologic lesion in familial steroid-resistant nephrotic syndrome, for which there is currently no treatment. Dysfunction of the glomerular podocyte, a specialized cell that forms the ... ...

    Abstract Focal Segmental Glomerulosclerosis (FSGS) is the typical renal histologic lesion in familial steroid-resistant nephrotic syndrome, for which there is currently no treatment. Dysfunction of the glomerular podocyte, a specialized cell that forms the glomerular filtration barrier, is central in the pathogenesis of FSGS. Here, we reported the generation of two isogenic iPS cell lines from a patient affected by FSGS, carrying the c.565G > A mutation in the PAX2 gene. The iPS cell lines we generated expressed pluripotency markers at the mRNA and protein levels and differentiated into all three germ layers. These iPSCs will be instrumental in understanding FSGS pathogenesis.
    MeSH term(s) Glomerulosclerosis, Focal Segmental/genetics ; Heterozygote ; Humans ; Induced Pluripotent Stem Cells/metabolism ; Male ; Mutation ; PAX2 Transcription Factor/genetics
    Chemical Substances PAX2 Transcription Factor ; PAX2 protein, human
    Language English
    Publishing date 2018-10-29
    Publishing country England
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1876-7753
    ISSN (online) 1876-7753
    DOI 10.1016/j.scr.2018.10.018
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  8. Article ; Online: Empagliflozin protects glomerular endothelial cell architecture in experimental diabetes through the VEGF-A/caveolin-1/PV-1 signaling pathway.

    Locatelli, Monica / Zoja, Carlamaria / Conti, Sara / Cerullo, Domenico / Corna, Daniela / Rottoli, Daniela / Zanchi, Cristina / Tomasoni, Susanna / Remuzzi, Giuseppe / Benigni, Ariela

    The Journal of pathology

    2022  Volume 256, Issue 4, Page(s) 468–479

    Abstract: In addition to having blood glucose-lowering effects, inhibitors of sodium glucose cotransporter 2 (SGLT2) afford renoprotection in diabetes. We sought to investigate which components of the glomerular filtration barrier could be involved in the ... ...

    Abstract In addition to having blood glucose-lowering effects, inhibitors of sodium glucose cotransporter 2 (SGLT2) afford renoprotection in diabetes. We sought to investigate which components of the glomerular filtration barrier could be involved in the antiproteinuric and renoprotective effects of SGLT2 inhibition in diabetes. BTBR (black and tan, brachyuric) ob/ob mice that develop a type 2 diabetic nephropathy received a standard diet with or without empagliflozin for 10 weeks, starting at 8 weeks of age, when animals had developed albuminuria. Empagliflozin caused marked decreases in blood glucose levels and albuminuria but did not correct glomerular hyperfiltration. The protective effect of empagliflozin against albuminuria was not due to a reduction in podocyte damage as empagliflozin did not affect the larger podocyte filtration slit pore size nor the defective expression of nephrin and nestin. Empagliflozin did not reduce the thickening of the glomerular basement membrane. In BTBR ob/ob mice, the most profound abnormality seen using electron microscopy was in the endothelial aspect of the glomerular capillary, with significant loss of endothelial fenestrations. Remarkably, empagliflozin ameliorated the subverted microvascular endothelial ultrastructure. Caveolae and bridging diaphragms between adjacent endothelial fenestrae were seen in diabetic mice and associated with increased expression of caveolin-1 and the appearance of PV-1. These endothelial abnormalities were limited by the SGLT2 inhibitor. Although no expression of SGLT2 was found in glomerular endothelial cells, SGLT2 was expressed in the podocytes of diabetic mice. VEGF-A, which is a known stimulus for endothelial caveolin-1 and PV-1, was increased in podocytes of BTBR ob/ob mice and normalized by SGLT2 inhibitor treatment. Thus, empagliflozin's protective effect on the glomerular endothelium of diabetic mice could be due to a limitation of the paracrine signaling of podocyte-derived VEGF-A that resulted in a reduction of the abnormal endothelial caveolin-1 and PV-1, with the consequent preservation of glomerular endothelial function and permeability. © 2022 The Pathological Society of Great Britain and Ireland.
    MeSH term(s) Albuminuria/drug therapy ; Albuminuria/pathology ; Albuminuria/prevention & control ; Animals ; Benzhydryl Compounds ; Blood Glucose/metabolism ; Caveolin 1/metabolism ; Diabetes Mellitus, Experimental/drug therapy ; Diabetic Nephropathies/drug therapy ; Diabetic Nephropathies/metabolism ; Diabetic Nephropathies/prevention & control ; Endothelial Cells/metabolism ; Female ; Glomerular Basement Membrane/metabolism ; Glucosides ; Humans ; Male ; Mice ; Signal Transduction ; Sodium-Glucose Transporter 2/metabolism ; Sodium-Glucose Transporter 2 Inhibitors/pharmacology ; Vascular Endothelial Growth Factor A/metabolism
    Chemical Substances Benzhydryl Compounds ; Blood Glucose ; Caveolin 1 ; Glucosides ; Sodium-Glucose Transporter 2 ; Sodium-Glucose Transporter 2 Inhibitors ; Vascular Endothelial Growth Factor A ; empagliflozin (HDC1R2M35U)
    Language English
    Publishing date 2022-02-23
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 3119-7
    ISSN 1096-9896 ; 0022-3417
    ISSN (online) 1096-9896
    ISSN 0022-3417
    DOI 10.1002/path.5862
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    In stock of ZB MED Cologne/Königswinter

    Ud II Zs.12: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  9. Article ; Online: Generation of two isogenic knockout PKD2 iPS cell lines, IRFMNi003-A-1 and IRFMNi003-A-2, using CRISPR/Cas9 technology.

    Trionfini, Piera / Ciampi, Osele / Romano, Elena / Benigni, Ariela / Tomasoni, Susanna

    Stem cell research

    2019  Volume 42, Page(s) 101667

    Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent inherited renal disease, characterized by multiple cysts that can lead to kidney failure resulting in end-stage renal disease. ADPKD is mainly caused by mutations in either the ... ...

    Abstract Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent inherited renal disease, characterized by multiple cysts that can lead to kidney failure resulting in end-stage renal disease. ADPKD is mainly caused by mutations in either the PKD1 and PKD2 genes, encoding for polycystin-1 and polycystin-2, respectively. In order to clarify the disease mechanisms, here we describe the generation of two isogenic induced pluripotent stem cell (iPSC) lines in which the PKD2 gene was deleted using CRISPR/Cas9 technology. The PKD2
    MeSH term(s) CRISPR-Cas Systems/genetics ; Cell Line ; Humans ; Induced Pluripotent Stem Cells/metabolism ; Mutation ; TRPP Cation Channels/genetics
    Chemical Substances TRPP Cation Channels ; polycystic kidney disease 2 protein
    Language English
    Publishing date 2019-11-29
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1876-7753
    ISSN (online) 1876-7753
    DOI 10.1016/j.scr.2019.101667
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Hypoimmunogenic Human Pluripotent Stem Cells as a Powerful Tool for Liver Regenerative Medicine.

    Trionfini, Piera / Romano, Elena / Varinelli, Marco / Longaretti, Lorena / Rizzo, Paola / Giampietro, Roberta / Caroli, Annalina / Aiello, Sistiana / Todeschini, Marta / Casiraghi, Federica / Remuzzi, Giuseppe / Benigni, Ariela / Tomasoni, Susanna

    International journal of molecular sciences

    2023  Volume 24, Issue 14

    Abstract: Induced pluripotent stem cells (iPSC) have huge potential as cell therapy for various diseases, given their potential for unlimited self-renewal and capability to differentiate into a wide range of cell types. Although autologous iPSCs represents the ... ...

    Abstract Induced pluripotent stem cells (iPSC) have huge potential as cell therapy for various diseases, given their potential for unlimited self-renewal and capability to differentiate into a wide range of cell types. Although autologous iPSCs represents the ideal source for patient-tailored regenerative medicine, the high costs of the extensive and time-consuming production process and the impracticability for treating acute conditions hinder their use for broad applications. An allogeneic iPSC-based strategy may overcome these issues, but it carries the risk of triggering an immune response. So far, several approaches based on genome-editing techniques to silence human leukocyte antigen class I (HLA-I) or II (HLA-II) expression have been explored to overcome the immune rejection of allogeneic iPSCs. In this study, we employed the CRISPR/Cas9 (clustered regularly interspaced short palindromic repeats/CRISPR associated protein 9) system to delete the β2-Microglobulin (
    MeSH term(s) Humans ; Regenerative Medicine ; Pluripotent Stem Cells ; Gene Editing/methods ; Induced Pluripotent Stem Cells ; Histocompatibility Antigens Class I/genetics ; Histocompatibility Antigens Class I/metabolism ; Liver
    Chemical Substances Histocompatibility Antigens Class I
    Language English
    Publishing date 2023-07-22
    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/ijms241411810
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