PMC Links for GEO Profiles (Select 8057922) - PMC

Links from GEO Profiles

Items: 1 to 20 of 50

<< First< Prev

Page of 3

Next >Last >>

Select item 109731421.

Defects of renal tubular homeostasis and cystogenesis in the Pkhd1 knockout

Julia C. Fox, Susanne T. Hahnenstein, Fatima Hassan, Andrea Grund, Dieter Haffner, Wolfgang H. Ziegler

iScience. 2024 Apr 19; 27(4): 109487. Published online 2024 Mar 11. doi: 10.1016/j.isci.2024.109487

PMCID:: PMC10973142

Abstract Article PDF–4.8M

Select item 109297482.

Cystin is required for maintaining fibrocystin (FPC) levels and safeguarding proteome integrity in mouse renal epithelial cells: A mechanistic connection between the kidney defects in cpk mice and human ARPKD

Yiming J. Zhang, Chaozhe Yang, Wei Wang, Naoe Harafuji, Piotr Stasiak, P. Darwin Bell, Ljubica Caldovic, Elizabeth Sztul, Lisa M. Guay‐Woodford, Zsuzsanna Bebok

FASEB J. 2023 Jul; 37(7): e23008. Published online 2023 Jun 15. doi: 10.1096/fj.202300100R

PMCID:: PMC10929748

Abstract Article PDF–5.9M

Select item 107314653.

Differential regulation of MYC expression by PKHD1/Pkhd1 in human and mouse kidneys: phenotypic implications for recessive polycystic kidney disease

Naoe Harafuji, Chaozhe Yang, Maoqing Wu, Girija Thiruvengadam, Heather Gordish-Dressman, R. Griffin Thompson, P. Darwin Bell, Avi Z. Rosenberg, Claudia Dafinger, Max C. Liebau, Zsuzsanna Bebok, Ljubica Caldovic, Lisa M. Guay-Woodford

Front Cell Dev Biol. 2023; 11: 1270980. Published online 2023 Nov 17. doi: 10.3389/fcell.2023.1270980

PMCID:: PMC10731465

Abstract Article PDF–2.4M

Select item 105793734.

Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and suppresses cystogenesis

Rebecca V Walker, Qin Yao, Hangxue Xu, Anthony Maranto, Kristen F Swaney, Sreekumar Ramachandran, Rong Li, Laura Cassina, Brian M Polster, Patricia Outeda, Alessandra Boletta, Terry Watnick, Feng Qian

Nat Commun. 2023; 14: 6513. Published online 2023 Oct 16. doi: 10.1038/s41467-023-42196-4

PMCID:: PMC10579373

Abstract Article PDF–12M

Select item 105296175.

Novel ARPKD mouse model with near complete deletion of Polycystic Kidney and Hepatic Disease 1 (Pkhd1) genomic locus has multiple phenotypes but no renal cysts

Yu Ishimoto, Luis F Menezes, Fang Zhou, Teruhiko Yoshida, Taishi Komori, Jiahe Qiu, Marian F Young, Huiyan Lu, Svetlana Potapova, Patricia Outeda, Terry Watnick, Gregory G Germino

Kidney Int. Author manuscript; available in PMC 2024 Sep 1.

Published in final edited form as: Kidney Int. 2023 Sep; 104(3): 611–616. Published online 2023 Jul 5. doi: 10.1016/j.kint.2023.05.027

PMCID:: PMC10529617

Article PDF–967K

Select item 104827576.

Pkhd1^cyli/cyli mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease

Chaozhe Yang, Naoe Harafuji, Ljubica Caldovic, Weiying Yu, Ravindra Boddu, Surajit Bhattacharya, Hayk Barseghyan, Heather Gordish-Dressman, Oded Foreman, Zsuzsa Bebok, Eva M. Eicher, Lisa M. Guay-Woodford

J Mol Med (Berl) 2023; 101(9): 1141–1151. Published online 2023 Aug 16. doi: 10.1007/s00109-023-02351-2

PMCID:: PMC10482757

Abstract Article PDF–1.6M

Select item 94682417.

Mutated Pkhd1 alone is sufficient to cause autoimmune biliary disease on the nonobese diabetic (NOD) genetic background

David E. Adams, Luke S. Heuer, Manuel Rojas, Weici Zhang, William M. Ridgway

Immunogenetics. 2023; 75(1): 27–37. Published online 2022 Sep 13. doi: 10.1007/s00251-022-01276-3

PMCID:: PMC9468241

Abstract Article PDF–2.1M

Select item 91508628.

Dysregulation of the Scribble/YAP/β-catenin axis sustains the fibroinflammatory response in a PKHD1^−/− mouse model of Congenital Hepatic Fibrosis

Luca Fabris, Chiara Milani, Romina Fiorotto, Valeria Mariotti, Eleanna Kaffe, Barbara Seller, Aurelio Sonzogni, Mario Strazzabosco, Massimiliano Cadamuro

FASEB J. Author manuscript; available in PMC 2023 Jun 1.

Published in final edited form as: FASEB J. 2022 Jun; 36(6): e22364. doi: 10.1096/fj.202101924R

PMCID:: PMC9150862

Abstract Article PDF–2.8M

Select item 85293989.

Therapeutic Potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease

Murali K. Yanda, Vartika Tomar, Liudmila Cebotaru

Cell Mol Gastroenterol Hepatol. 2021; 12(5): 1517–1529. Published online 2021 Jul 27. doi: 10.1016/j.jcmgh.2021.07.012

PMCID:: PMC8529398

Abstract Article PDF–4.8M

Select item 817039910.

Down-Regulation of Inpp5e Associated With Abnormal Ciliogenesis During Embryonic Neurodevelopment Under Inositol Deficiency

Huixuan Yue, Shen Li, Jiaxing Qin, Tingting Gao, Jianjun Lyu, Yu Liu, Xiuwei Wang, Zhen Guan, Zhiqiang Zhu, Bo Niu, Rugang Zhong, Jin Guo, Jianhua Wang

Front Neurol. 2021; 12: 579998. Published online 2021 May 19. doi: 10.3389/fneur.2021.579998

PMCID:: PMC8170399

Abstract Article PDF–3.0M

Select item 812647911.

Hnf1b haploinsufficiency differentially affects developmental target genes in a new renal cysts and diabetes mouse model

Leticia L. Niborski, Mélanie Paces-Fessy, Pierbruno Ricci, Adeline Bourgeois, Pedro Magalhães, Maria Kuzma-Kuzniarska, Celine Lesaulnier, Martin Reczko, Edwige Declercq, Petra Zürbig, Alain Doucet, Muriel Umbhauer, Silvia Cereghini

Dis Model Mech. 2021 May 1; 14(5): dmm047498. Published online 2021 May 4. doi: 10.1242/dmm.047498

PMCID:: PMC8126479

Abstract Article

Select item 644237712.

Heterozygous Pkhd1^C642* mice develop cystic liver disease and proximal tubule ectasia that mimics radiographic signs of medullary sponge kidney

Dan Shan, Gabriel Rezonzew, Sean Mullen, Ronald Roye, Juling Zhou, Phillip Chumley, Dustin Z. Revell, Anil Challa, Harrison Kim, Mark E. Lockhart, Trenton R. Schoeb, Mandy J. Croyle, Robert A. Kesterson, Bradley K. Yoder, Lisa M. Guay-Woodford, Michal Mrug

Am J Physiol Renal Physiol. 2019 Mar 1; 316(3): F463–F472. Published online 2019 Jan 2. doi: 10.1152/ajprenal.00181.2018

PMCID:: PMC6442377

Abstract Article

Select item 633544013.

Atmin modulates Pkhd1 expression and may mediate Autosomal Recessive Polycystic Kidney Disease (ARPKD) through altered non-canonical Wnt/Planar Cell Polarity (PCP) signalling

Taylor Richards, Kavindiya Modarage, Charlotte Dean, Aidan McCarthy-Boxer, Helen Hilton, Chris Esapa, Jill Norman, Patricia Wilson, Paraskevi Goggolidou

Biochim Biophys Acta Mol Basis Dis. 2019 Feb 1; 1865(2): 378–390. doi: 10.1016/j.bbadis.2018.11.003

PMCID:: PMC6335440

Abstract Article

Select item 600517314.

A novel model of autosomal recessive polycystic kidney questions the role of the Fibrocystin C-terminus in disease mechanism

Patricia Outeda, Luis Menezes, Erum A. Hartung, Stacey Bridges, Fang Zhou, Xianjun Zhu, Hangxue Xu, Qiong Huang, Qin Yao, Feng Qian, Gregory G Germino, Terry Watnick

Kidney Int. Author manuscript; available in PMC 2018 Nov 1.

Published in final edited form as: Kidney Int. 2017 Nov; 92(5): 1130–1144. Published online 2017 Jul 18. doi: 10.1016/j.kint.2017.04.027

PMCID:: PMC6005173

Abstract Article PDF–4.2M

Select item 575751115.

A Novel Pkhd1 mutation interacts with the Nonobese diabetic (NOD) genetic background to cause autoimmune cholangitis

Wenting Huang, Daniel B. Rainbow, Yuehong Wu, David Adams, Pranavkumar Shivakumar, Leah Kottyan, Rebekah Karns, Bruce Aronow, Jorge Bezerra, M. Eric Gershwin, Laurence B. Peterson, Linda S. Wicker, William M Ridgway

J Immunol. Author manuscript; available in PMC 2018 Jul 1.

Published in final edited form as: J Immunol. 2018 Jan 1; 200(1): 147–162. Published online 2017 Nov 20. doi: 10.4049/jimmunol.1701087

PMCID:: PMC5757511

Abstract Article PDF–999K

Select item 545084016.

Embryonic cholecystitis and defective gallbladder contraction in the Sox17-haploinsufficient mouse model of biliary atresia

Hiroki Higashiyama, Aisa Ozawa, Hiroyuki Sumitomo, Mami Uemura, Ko Fujino, Hitomi Igarashi, Kenya Imaimatsu, Naoki Tsunekawa, Yoshikazu Hirate, Masamichi Kurohmaru, Yukio Saijoh, Masami Kanai-Azuma, Yoshiakira Kanai

Development. 2017 May 15; 144(10): 1906–1917. doi: 10.1242/dev.147512

PMCID:: PMC5450840

Abstract Article

Select item 535051617.

Tubby family proteins are adapters for ciliary trafficking of integral membrane proteins

Hemant B. Badgandi, Sun-hee Hwang, Issei S. Shimada, Evan Loriot, Saikat Mukhopadhyay

J Cell Biol. 2017 Mar 6; 216(3): 743–760. doi: 10.1083/jcb.201607095

PMCID:: PMC5350516

Abstract Article PDF–3.1M

Select item 512391918.

Loss of Zeb2 in mesenchyme-derived nephrons causes primary glomerulocystic disease

Hila Milo Rasouly, Sudhir Kumar, Stefanie Chan, Anna Pisarek-Horowitz, Richa Sharma, Qiongchao J. Xi, Yuriko Nishizaki, Yujiro Higashi, David J. Salant, Richard L. Maas, Weining Lu

Kidney Int. Author manuscript; available in PMC 2017 Dec 1.

Published in final edited form as: Kidney Int. 2016 Dec; 90(6): 1262–1273. Published online 2016 Aug 31. doi: 10.1016/j.kint.2016.06.037

PMCID:: PMC5123919

Abstract Article PDF–2.1M

Select item 476446019.

MACROPHAGE RECRUITMENT BY FIBROCYSTIN-DEFECTIVE BILIARY EPITHELIAL CELLS PROMOTES PORTAL FIBROSIS IN CONGENITAL HEPATIC FIBROSIS

Luigi Locatelli, Massimiliano Cadamuro, Carlo Spirli, Romina Fiorotto, Silvia Lecchi, Carola Maria Morell, Yury Popov, Roberto Scirpo, Maria De Matteis, Mariangela Amenduni, Andrea Pietrobattista, Giuliano Torre, Detlef Schuppan, Luca Fabris, Mario Strazzabosco

Hepatology. Author manuscript; available in PMC 2017 Mar 1.

Published in final edited form as: Hepatology. 2016 Mar; 63(3): 965–982. Published online 2016 Jan 16. doi: 10.1002/hep.28382

PMCID:: PMC4764460

Abstract Article PDF–3.2M

Select item 435298120.

Hnf1b controls pancreas morphogenesis and the generation of Ngn3⁺ endocrine progenitors

Matias G. De Vas, Janel L. Kopp, Claire Heliot, Maike Sander, Silvia Cereghini, Cécile Haumaitre

Development. 2015 Mar 1; 142(5): 871–882. doi: 10.1242/dev.110759

PMCID:: PMC4352981

Abstract Article

PMC

Result Filters

Article attributes

Text availability

Publication date

Custom date range

Research Funder

Additional filters

Display Settings:

Send to:

Links from GEO Profiles

Items: 1 to 20 of 50

Display Settings:

Send to:

Supplemental Content

Find related data

Recent activity