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  1. Article ; Online: Loss of Cilia Does Not Slow Liver Disease Progression in Mouse Models of Autosomal Recessive Polycystic Kidney Disease.

    Gallagher, Anna Rachel / Somlo, Stefan

    Kidney360

    2021  Volume 1, Issue 9, Page(s) 962–968

    MeSH term(s) Animals ; Cilia ; Disease Progression ; Liver ; Mice ; Polycystic Kidney, Autosomal Recessive/genetics ; TRPP Cation Channels
    Chemical Substances TRPP Cation Channels
    Language English
    Publishing date 2021-04-07
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ISSN 2641-7650
    ISSN (online) 2641-7650
    DOI 10.34067/kid.0001022019
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Developing and implementing survivor-centred approaches for college student-athletes: perspectives from athletic department, Title IX, and campus advocacy personnel.

    Bender, Anna E / Adhia, Avanti / Ross, Rachel / Gallagher, Amy / Mustafa, Ayah / Kroshus, Emily / Ellyson, Alice M

    European journal of psychotraumatology

    2024  Volume 15, Issue 1, Page(s) 2334587

    Abstract: ... ...

    Abstract ABSTRACT
    MeSH term(s) Humans ; Female ; Adult ; Sex Offenses ; Sports ; Students ; Athletes ; Survivors
    Language English
    Publishing date 2024-04-08
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2586642-4
    ISSN 2000-8066 ; 2000-8066
    ISSN (online) 2000-8066
    ISSN 2000-8066
    DOI 10.1080/20008066.2024.2334587
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Adult Inactivation of the Recessive Polycystic Kidney Disease Gene Causes Polycystic Liver Disease.

    Besse, Whitney / Roosendaal, Charlotte / Tuccillo, Luigi / Roy, Sounak Ghosh / Gallagher, Anna-Rachel / Somlo, Stefan

    Kidney360

    2021  Volume 1, Issue 10, Page(s) 1068–1076

    Abstract: Background: A major difference between autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD) lies in the pattern of inheritance, and the resultant timing and focality of cyst formation. In both ... ...

    Abstract Background: A major difference between autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD) lies in the pattern of inheritance, and the resultant timing and focality of cyst formation. In both diseases, cysts form in the kidney and liver as a consequence of the cellular recessive genotype of the respective disease gene, but this occurs by germline inheritance in ARPKD and somatic second hit mutations to the one normal allele in ADPKD. The fibrocystic liver phenotype in ARPKD is attributed to abnormal ductal plate formation because of the absence of
    Methods: We used an adult-inducible
    Results: Inactivation of
    Conclusions: Somatic adult inactivation of
    MeSH term(s) Animals ; Cysts/genetics ; Female ; Liver Diseases/genetics ; Mice ; Polycystic Kidney, Autosomal Recessive/genetics ; Receptors, Cell Surface/genetics
    Chemical Substances Pkhd1 protein, mouse ; Receptors, Cell Surface
    Language English
    Publishing date 2021-02-04
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ISSN 2641-7650
    ISSN (online) 2641-7650
    DOI 10.34067/kid.0002522020
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Ciliary Mechanisms of Cyst Formation in Polycystic Kidney Disease.

    Ma, Ming / Gallagher, Anna-Rachel / Somlo, Stefan

    Cold Spring Harbor perspectives in biology

    2017  Volume 9, Issue 11

    Abstract: Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled sacs called cysts. The causal ... ...

    Abstract Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled sacs called cysts. The causal genes
    MeSH term(s) Animals ; Cilia/physiology ; Cysts/pathology ; Hedgehog Proteins/metabolism ; Homeostasis ; Humans ; Polycystic Kidney, Autosomal Dominant/pathology ; Signal Transduction
    Chemical Substances Hedgehog Proteins
    Language English
    Publishing date 2017-11-01
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 1943-0264
    ISSN (online) 1943-0264
    DOI 10.1101/cshperspect.a028209
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: A synthetic agent ameliorates polycystic kidney disease by promoting apoptosis of cystic cells through increased oxidative stress.

    Fedeles, Bogdan I / Bhardwaj, Rishi / Ishikawa, Yasunobu / Khumsubdee, Sakunchai / Krappitz, Matteus / Gubina, Nina / Volpe, Isabel / Andrade, Denise C / Westergerling, Parisa / Staudner, Tobias / Campolo, Jake / Liu, Sally S / Dong, Ke / Cai, Yiqiang / Rehman, Michael / Gallagher, Anna-Rachel / Ruchirawat, Somsak / Croy, Robert G / Essigmann, John M /
    Fedeles, Sorin V / Somlo, Stefan

    Proceedings of the National Academy of Sciences of the United States of America

    2024  Volume 121, Issue 4, Page(s) e2317344121

    Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of chronic kidney disease and the fourth leading cause of end-stage kidney disease, accounting for over 50% of prevalent cases requiring renal replacement therapy. ... ...

    Abstract Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of chronic kidney disease and the fourth leading cause of end-stage kidney disease, accounting for over 50% of prevalent cases requiring renal replacement therapy. There is a pressing need for improved therapy for ADPKD. Recent insights into the pathophysiology of ADPKD revealed that cyst cells undergo metabolic changes that up-regulate aerobic glycolysis in lieu of mitochondrial respiration for energy production, a process that ostensibly fuels their increased proliferation. The present work leverages this metabolic disruption as a way to selectively target cyst cells for apoptosis. This small-molecule therapeutic strategy utilizes 11beta-dichloro, a repurposed DNA-damaging anti-tumor agent that induces apoptosis by exacerbating mitochondrial oxidative stress. Here, we demonstrate that 11beta-dichloro is effective in delaying cyst growth and its associated inflammatory and fibrotic events, thus preserving kidney function in perinatal and adult mouse models of ADPKD. In both models, the cyst cells with homozygous inactivation of
    MeSH term(s) Mice ; Animals ; Polycystic Kidney, Autosomal Dominant/drug therapy ; Polycystic Kidney, Autosomal Dominant/genetics ; Polycystic Kidney, Autosomal Dominant/metabolism ; Cell Proliferation ; Polycystic Kidney Diseases/metabolism ; Apoptosis ; Oxidative Stress ; Cysts/metabolism ; DNA/metabolism ; Kidney/metabolism ; TRPP Cation Channels/genetics
    Chemical Substances DNA (9007-49-2) ; TRPP Cation Channels
    Language English
    Publishing date 2024-01-19
    Publishing country United States
    Document type Journal Article
    ZDB-ID 209104-5
    ISSN 1091-6490 ; 0027-8424
    ISSN (online) 1091-6490
    ISSN 0027-8424
    DOI 10.1073/pnas.2317344121
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1148: 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: Is it Time to Fold the Cysts Away?

    Krappitz, Matteus / Gallagher, Anna-Rachel / Fedeles, Sorin

    Trends in molecular medicine

    2016  Volume 22, Issue 12, Page(s) 997–999

    Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 and PKD2, encoding polycystin-1 and polycystin-2, respectively. Optimizing the folding environment for polycystin-1 missense mutations may have a critical effect on the ... ...

    Abstract Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 and PKD2, encoding polycystin-1 and polycystin-2, respectively. Optimizing the folding environment for polycystin-1 missense mutations may have a critical effect on the progression of ADPKD in animal models and could potentially lead to tangible therapeutic options for subgroups of ADPKD patients.
    MeSH term(s) Animals ; Drug Discovery ; Humans ; Mutation, Missense/drug effects ; Polycystic Kidney, Autosomal Dominant/drug therapy ; Polycystic Kidney, Autosomal Dominant/genetics ; Protein Folding/drug effects ; TRPP Cation Channels/chemistry ; TRPP Cation Channels/genetics
    Chemical Substances TRPP Cation Channels ; polycystic kidney disease 1 protein ; polycystic kidney disease 2 protein
    Language English
    Publishing date 2016-12
    Publishing country England
    Document type Journal Article
    ZDB-ID 2036490-8
    ISSN 1471-499X ; 1471-4914
    ISSN (online) 1471-499X
    ISSN 1471-4914
    DOI 10.1016/j.molmed.2016.10.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 4345: Show issues Location:
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    Jg. 1995 - 2021: Lesesall (2.OG)
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  7. Article ; Online: XBP1 Activation Reduces Severity of Polycystic Kidney Disease due to a Nontruncating Polycystin-1 Mutation in Mice.

    Krappitz, Matteus / Bhardwaj, Rishi / Dong, Ke / Staudner, Tobias / Yilmaz, Duygu Elif / Pioppini, Carlotta / Westergerling, Parisa / Ruemmele, David / Hollmann, Till / Nguyen, Thuy Anh / Cai, Yiqiang / Gallagher, Anna-Rachel / Somlo, Stefan / Fedeles, Sorin

    Journal of the American Society of Nephrology : JASN

    2022  Volume 34, Issue 1, Page(s) 110–121

    Abstract: Background: Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in Pkd1 and Pkd2. They encode the polytopic integral membrane proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively, which are expressed on primary cilia. ...

    Abstract Background: Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in Pkd1 and Pkd2. They encode the polytopic integral membrane proteins polycystin-1 (PC1) and polycystin-2 (PC2), respectively, which are expressed on primary cilia. Formation of kidney cysts in ADPKD starts when a somatic second hit mechanism inactivates the wild-type Pkd allele. Approximately one quarter of families with ADPDK due to Pkd1 have germline nonsynonymous amino acid substitution (missense) mutations. A subset of these mutations is hypomorphic, retaining some residual PC1 function. Previous studies have shown that the highly conserved Ire1 α -XBP1 pathway of the unfolded protein response can modulate levels of functional PC1 in the presence of mutations in genes required for post-translational maturation of integral membrane proteins. We examine how activity of the endoplasmic reticulum chaperone-inducing transcription factor XBP1 affects ADPKD in a murine model with missense Pkd1 .
    Methods: We engineered a Pkd1 REJ domain missense murine model, Pkd1 R2216W , on the basis of the orthologous human hypomorphic allele Pkd1 R2220W , and examined the effects of transgenic activation of XBP1 on ADPKD progression.
    Results: Expression of active XBP1 in cultured cells bearing PC1 R2216W mutations increased levels and ciliary trafficking of PC1 R2216W . Mice homozygous for Pkd1 R2216W or heterozygous for Pkd1 R2216Win trans with a conditional Pkd1 fl allele exhibit severe ADPKD following inactivation in neonates or adults. Transgenic expression of spliced XBP1 in tubule segments destined to form cysts reduced cell proliferation and improved Pkd progression, according to structural and functional parameters.
    Conclusions: Modulating ER chaperone function through XBP1 activity improved Pkd in a murine model of PC1, suggesting therapeutic targeting of hypomorphic mutations.
    MeSH term(s) Adult ; Mice ; Humans ; Animals ; Polycystic Kidney, Autosomal Dominant/metabolism ; TRPP Cation Channels/genetics ; TRPP Cation Channels/metabolism ; Disease Models, Animal ; Polycystic Kidney Diseases/metabolism ; Mutation ; X-Box Binding Protein 1/genetics ; X-Box Binding Protein 1/metabolism
    Chemical Substances TRPP Cation Channels ; XBP1 protein, human ; X-Box Binding Protein 1
    Language English
    Publishing date 2022-10-21
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 1085942-1
    ISSN 1533-3450 ; 1046-6673
    ISSN (online) 1533-3450
    ISSN 1046-6673
    DOI 10.1681/ASN.2021091180
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 3344: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (2.OG)
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  8. Article ; Online: Spliced XBP1 Rescues Renal Interstitial Inflammation Due to Loss of

    Ishikawa, Yasunobu / Fedeles, Sorin / Marlier, Arnaud / Zhang, Chao / Gallagher, Anna-Rachel / Lee, Ann-Hwee / Somlo, Stefan

    Journal of the American Society of Nephrology : JASN

    2019  Volume 30, Issue 3, Page(s) 443–459

    Abstract: Background: SEC63: Methods: We explored the renal effects of postnatal inactivation of : Results: The later onset of inactivation of : Conclusions: In the absence ... ...

    Abstract Background: SEC63
    Methods: We explored the renal effects of postnatal inactivation of
    Results: The later onset of inactivation of
    Conclusions: In the absence of
    Language English
    Publishing date 2019-02-11
    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.2018060614
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 3344: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (2.OG)
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  9. Article ; Online: Cell polarity and cystic kidney disease.

    Fedeles, Sorin / Gallagher, Anna Rachel

    Pediatric nephrology (Berlin, Germany)

    2012  Volume 28, Issue 8, Page(s) 1161–1172

    Abstract: Epithelial cell polarity is essential for organ development; aberrations in this process have been implicated in various diseases, including polycystic kidney disease. Establishment and maintenance of cell polarity is governed by a number of molecular ... ...

    Abstract Epithelial cell polarity is essential for organ development; aberrations in this process have been implicated in various diseases, including polycystic kidney disease. Establishment and maintenance of cell polarity is governed by a number of molecular processes and how these processes operate remains an interesting question. Conserved protein complexes guide both apical-basolateral polarity and planar cell polarity. In this review we discuss the recent findings that provide insights into polarity mechanisms and the intriguing crosstalk between apical-basolateral polarity and planar cell polarity, and their relationship to cystic kidney disease.
    MeSH term(s) Animals ; Cell Polarity ; Cilia/metabolism ; Cilia/pathology ; Epithelial Cells/metabolism ; Epithelial Cells/pathology ; Humans ; Intercellular Junctions/metabolism ; Intercellular Junctions/pathology ; Kidney/metabolism ; Kidney/pathology ; Kidney Diseases, Cystic/metabolism ; Kidney Diseases, Cystic/pathology ; Signal Transduction
    Language English
    Publishing date 2012-11-16
    Publishing country Germany
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-012-2337-z
    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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    Jg. 1995 - 2021: Lesesall (1.OG)
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  10. Article ; Online: A Fibrillating Left Atrial Appendage During Sinus Rhythm.

    Harding, Idris / Bastiaenen, Rachel / Khan, Parisha / Marciniak, Anna / Sharma, Rajan / Gallagher, Mark M

    JACC. Clinical electrophysiology

    2017  Volume 3, Issue 5, Page(s) 524–525

    MeSH term(s) Anticoagulants/therapeutic use ; Atrial Appendage/diagnostic imaging ; Atrial Appendage/physiology ; Atrial Fibrillation/diagnostic imaging ; Atrial Fibrillation/physiopathology ; Atrial Fibrillation/surgery ; Atrioventricular Block/diagnostic imaging ; Atrioventricular Block/etiology ; Atrioventricular Block/physiopathology ; Catheter Ablation ; Echocardiography, Doppler, Pulsed ; Echocardiography, Transesophageal ; Female ; Humans ; Middle Aged ; Recurrence ; Reoperation
    Chemical Substances Anticoagulants
    Language English
    Publishing date 2017-02-01
    Publishing country United States
    Document type Case Reports ; Journal Article ; Video-Audio Media
    ZDB-ID 2846739-5
    ISSN 2405-5018 ; 2405-500X ; 2405-500X
    ISSN (online) 2405-5018 ; 2405-500X
    ISSN 2405-500X
    DOI 10.1016/j.jacep.2016.09.020
    Database MEDical Literature Analysis and Retrieval System OnLINE

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