2020

Zimmerman KA, Hopp K, Mrug M. Role of chemokines, innate and adaptive immunity. Cell Signal. 2020 Sep;73:109647. doi: 10.1016/j.cellsig.2020.109647. Epub 2020 Apr 20. PMID: 32325183 

Seliger SL, Watnick T, Althouse AD, Perrone RD, Abebe KZ, Hallows KR, Miskulin DC, Bae KT. Baseline Characteristics and Patient-Reported Outcomes of ADPKD Patients in the Multicenter TAME-PKD Clinical Trial. Kidney360. 2020 Dec 31;1(12):1363-1372. doi: 10.34067/KID.0004002020. PMID: 33768205

Ha K, Nobuhara M, Wang Q, et al. The heteromeric PC-1/PC-2 polycystin complex is activated by the PC-1 N-terminus.  Elife. 2020 Nov 9;9:e60684. doi: 10.7554/eLife.60684. PMID: 33164752. 

2021

Jung HJ, Coleman R, Woodward OM et al (2021) Doxycycline Changes the Transcriptome Profile of mIMCD3 Renal Epithelial Cells. Frontiers in Physiology Renal and Epithelial Physiology. Nov 5;12:771691. doi: 10.3389/fphys.2021.771691.  PMCID: PMC8602682

Cordido A, Nuñez-Gonzalez L, Martinez-Moreno JM, et al. TWEAK Signaling Pathway Blockade Slows Cyst Growth and Disease Progression in Autosomal Dominant Polycystic Kidney Disease J Am Soc Nephrol. 2021 Aug;32(8):1913-1932. doi: 10.1681/ASN.2020071094. Epub 2021 Jun 21.  PMID: 34155062

Aloria EJG, Song CJ, Li et al. Ly6chi Infiltrating Macrophages Promote Cyst Progression in Injured Conditional Ift88 Mice. Kidney 360 24: 989-95, 2021.

Senum SR, Li YSM, Benson KA, Joli G, Olinger E, Lavu S, Madsen CD, Gregory AV, Neatu R, Kline TL, Audrézet MP, Outeda P, Nau CB, Meijer E, Ali H, Steinman TI, Mrug M, Phelan PJ, Watnick TJ, Peters DJM, Ong ACM, Conlon PJ, Perrone RD, Cornec-Le Gall E, Hogan MC, Torres VE, Sayer JA; Genomics England Research Consortium, the HALT PKD, CRISP, DIPAK, ADPKD Modifier, and TAME PKD studies, Harris PC. Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype. Am J Hum Genet. 2022 Jan 6;109(1):136-156. doi: 10.1016/j.ajhg.2021.11.016. Epub 2021 Dec 9. PMID: 34890546

Perrone RD, Abebe KZ, Watnick TJ, Althouse AD, Hallows KR, Lalama CM, Miskulin DC, Seliger SL, Tao C, Harris PC, Bae KT. Primary results of the randomized trial of metformin administration in polycystic kidney disease (TAME PKD). Kidney Int. 2021 Sep;100(3):684-696. doi: 10.1016/j.kint.2021.06.013. Epub 2021 Jun 27. PMID: 34186056.

Yao Q, Outeda P, Xu H, Walker R, Basquin D, Qian F, Cebotaru L, Watnick T, Cebotaru V. Polycystin-1 dependent regulation of polycystin-2 via GRP94, a member of HSP90 family that resides in the endoplasmic reticulum. FASEB J. 2021 Oct;35(10):e21865. doi: 10.1096/fj.202100325RR. PMID: 34486178

Yanda MK, Tomar V, Cole R et al. The Mitochondrial Ca2+ import complex is altered in ADPKD. Cell Calcium. 2022 Jan;101:102501. doi: 10.1016/j.ceca.2021.102501. Epub 2021 Nov 19.PMID: 34823104

Jamadar A, Suma SM, Mathew S et al. The tyrosine-kinase inhibitor Nintedanib ameliorates autosomal-dominant polycystic kidney disease. Cell Death Dis. 2021 Oct 14;12(10):947. PMID: 34650051.

El Ters M, Lu P, Mahnken JD et al.  Prognostic Value of Fibroblast Growth Factor 23 in Autosomal Dominant Polycystic Kidney Disease. Kidney Int Rep. 2021 Jan 16;6(4):953-961. doi: 10.1016/j.ekir.2021.01.004. PMID: 33912745.

Radadiya PS, Thornton MM, Daniel EA et al. Quinomycin A reduces cyst progression in polycystic kidney disease. FASEB J. 2021 May;35(5):e21533. doi: 10.1096/fj.202002490R. PMID: 33826787.

Radadiya PS, Thornton MM, Puri RV et al. Ciclopirox olamine induces ferritinophagy and reduces cyst burden in polycystic kidney disease. JCI Insight. 2021 Mar 30;6(8):e141299. doi: 10.1172/jci.insight.141299.PMID: 33784251

Chen C, Xu Q, Zhang Y, Davies BA, Huang Y, Katzmann DJ, Harris PC, Hu J, Ling K. Ciliopathy protein HYLS1 coordinates the biogenesis and signaling of primary cilia by activating the ciliary lipid kinase PIPKIγ. Sci Adv. 2021 Jun 23;7(26):eabe3401. doi: 10.1126/sciadv.abe3401. Print 2021 Jun.  PMID: 34162535

Ramalingam H, Kashyap S, Cobo-Stark P, Flaten A, Chang CM, Hajarnis S, Hein KZ, Lika J, Warner GM, Espindola-Netto JM, Kumar A, Kanchwala M, Xing C, Chini EN, Patel V. A methionine-Mettl3-N6-methyladenosine axis promotes polycystic kidney disease. Cell Metab. 2021 Jun 1;33(6):1234-1247.e7. doi: 10.1016/j.cmet.2021.03.024. Epub 2021 Apr 13. PMID: 33852874

Hopp K, Catenacci VA, Dwivedi N, Kline TL, Wang W, You Z, Nguyen DT, Bing K, Poudyal B, Johnson GC, Jackman MR, Miller M, Steele CN, Serkova NJ, MacLean PS, Nemenoff RA, Gitomer B, Chonchol M, Nowak KL. Weight loss and cystic disease progression in autosomal dominant polycystic kidney disease. iScience. 2021 Dec 27;25(1):103697.  PMID: 35059607.

2022

Choudhury MI, Li Y, Mistriotis P et al. Nat Commun. 2022 Apr 28;13(1):2317. doi: 10.1038/s41467-022-29988-w. PMID: 35484146  

Cordido A, Gonzalez-Vizoso C, Nuñez-Gonzalez L, et al. Quantitative Proteomic Study Unmasks Fibrinogen Pathway in Polycystic Liver Disease. Biomedicines 10:290, 2022 doi: 10.3390/biomedicines10020290. PMID: 35203500 PMCID: PMC8869147

Song CJ, Li Zhang, Ummey KBA et al. A Comprehensive Immune Cell Atlas of Cystic Kidney Disease Reveals the Involvement of Adaptive Immune Cells in Injury-Mediated Cyst Progression in Mice. J Am Soc Neprol 33:747-68, 2022.

Amaral AG, da Silva CCC, Serna JDC et al. Disruption of polycystin-1 cleavage leads to cardiac metabolic rewiring in mice. Biochim Biophys Acta Mol Basis Dis. 2022 Jun 1;1868(6):166371. doi: 10.1016/j.bbadis.2022.166371. Epub 2022 Feb 24. PMID: 35218894. 

Lakhia, R, Ramalingam H, Chang C-M et al. PKD1 and PKD2 mRNA cis-inhibition drives polycystic kidney disease progression. Nat Commun 13, 4765 (2022). https://doi.org/10.1038/s41467-022-32543-2. PMID: 35965273 PMCID: PMC9376183

Muto Y, Dixon EE, Yoshimura Y et al. Defining cellular complexity in human autosomal dominant polycystic kidney disease by multimodal single cell analysis. Nat Commun 13, 6497 (2022). https://doi.org/10.1038/s41467-022-34255-z. PMID: 36310237

Walker RV, Maranto A, Palicharla VR et al. Cilia-Localized Counterregulatory Signals as Drivers of Renal Cystogenesis. Front. Mol Biosci 2022 Jun 23;9:936070.  doi:10.3389/fmolb.2022.936070. eCollection 2022. PMID: 35832738.

Riddle HAL, Zhang S, Qian F et al. Kidney stone formation in a novel murine model of polycystic kidney disease. Am J Physiol Renal Physiol 323: F59-68, 2022. PMID: 35343849 PMCID: PMC9236864.

Pawnikar S, Magenheimer BS, Munoz EN et al. Mechanism of tethered agonist-mediated signaling by polycystin-1. Proc Natl Acad Sci U S A 2022 May 10;119(19):e2113786119. doi: 10.1073/pnas.2113786119. PMID: 35522707 PMCID: PMC9171645.

Zhang Y, Daniel EA, Metcalf J et al. CaMK4 overexpression in polycystic kidney disease promotes mTOR-mediated cell proliferation. Mol Cell Biol 2022 Aug 24;mjac050. doi: 10.1093/jmcb/mjac050. PMID: 36002021.

Sundar SV, Zhou JX, Magenheimer BS et al. The lonidamine derivative H2-gamendazole reduces cyst formation in polycystic kidney disease. Am J Physiol Renal Physiol 2022 Oct 1;323(4):F492-F506.  PMID: 35979967 PMCID: PMC9529276.

Wang W, Silva LM, Wang HH et al. Ttc21b deficiency attenuates autosomal dominant polycystic kidney disease in a kidney tubular- and maturation-dependent manner. Kidney Int. 102:577-591, 2022. PMID: 35644283.

MacKay CE, Floen M, Leo MD, Hasan R, Garrud TAC, Fernández-Peña C, Singh P, Malik KU, Jaggar JH. A plasma membrane-localized polycystin-1/polycystin-2 complex in endothelial cells elicits vasodilation. Elife. 2022 Mar 1;11:e74765. doi: 10.7554/eLife.74765. PMID: 35229718; PMCID: PMC8933003.

Hallows KR, Li H, Saitta B, Sepehr S, Huang P, Pham J, Wang J, Mancino V, Chung EJ, Pinkosky SL and Pastor-Soler NM (2022), Beneficial effects of bempedoic acid treatment in polycystic kidney disease cells and mice. Front. Mol. Biosci. 9:1001941. doi: 10.3389/fmolb.2022.1001941

Swenson-Fields KI, Ward CJ, Lopez ME, Fross S, Heimes Dillon AL, Meisenheimer JD, Rabbani AJ, Wedlock E, Basu MK, Jansson KP, Rowe PS, Stubbs JR, Wallace DP, Vitek MP, Fields TA. Caspase-1 and the inflammasome promote polycystic kidney disease progression. Front Mol Biosci. 2022 Nov 29;9:971219. doi: 10.3389/fmolb.2022.971219. eCollection 2022. PMID: 36523654

Wang J, Thomas HR, Thompson RG, Waldrep SC, Fogerty J, Song P, Li Z, Ma Y, Santra P, Hoover JD, Yeo NC, Drummond IA, Yoder BK, Amack JD, Perkins B, Parant JM.  Variable phenotypes and penetrance between and within different zebrafish ciliary transition zone mutants. Dis Model Mech. 2022 Dec 1;15(12):dmm049568. doi: 10.1242/dmm.049568. Epub 2022 Dec 19. PMID: 36533556

Bentley-Ford MR, LaBonty M, Thomas HR, Haycraft CJ, Scott M, LaFayette C, Croyle MJ, Andersen RS, Parant JM, Yoder BK. Evolutionarily conserved genetic interactions between nphp-4 and bbs-5 mutations exacerbate ciliopathy phenotypes. Genetics. 2022 Jan 4;220(1):iyab209. doi: 10.1093/genetics/iyab209. PMID: 34850872

Padhy B, Xie J, Wang R, Lin F, Huang CL. Channel Function of Polycystin-2 in the Endoplasmic Reticulum Protects against Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 2022 Aug;33(8):1501-1516. doi: 10.1681/ASN.2022010053. Epub 2022 Jul 14. PMID: 35835458

Chen C, Hu J, Ling K. The Role of Primary Cilia-Associated Phosphoinositide Signaling in Development. J Dev Biol. 2022 Dec 2;10(4):51. doi: 10.3390/jdb10040051. PMID: 36547473

Panda DK, Bai X, Zhang Y, Stylianesis NA, Koromilas AE, Lipman ML, Karaplis AC. SCF-SKP2 E3 ubiquitin ligase links mTORC1/ER stress/ISR with YAP activation in murine renal cystogenesis. J Clin Invest. 2022 Dec 15;132(24):e153943. doi: 10.1172/JCI153943. PMID: 36326820

Katsianou MA, Papavassiliou KA, Gargalionis AN, Agrogiannis G, Korkolopoulou P, Panagopoulos D, Themistocleous MS, Piperi C, Basdra EK, Papavassiliou AG. Polycystin-1 regulates cell proliferation and migration through AKT/mTORC2 pathway in a human craniosynostosis cell model. J Cell Mol Med. 2022 Apr;26(8):2428-2437. doi: 10.1111/jcmm.17266. Epub 2022 Mar 13. PMID: 35285136; PMCID: PMC8995461.

2023

Dustin T. Nguyen, … , Jelena Klawitter, Katharina Hopp et al. The tryptophan-metabolizing enzyme indoleamine 2,3-dioxygenase 1 regulates polycystic kidney disease progression. JCI Insight. 2023;8(1):e154773. https://doi.org/10.1172/jci.insight.154773.

Onuchic, L., Padovano, V., Schena, G. et al. The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion. Nat Commun 14, 1790 (2023). https://doi.org/10.1038/s41467-023-37449-1.

Nguyen DT, Kleczko EK, Dwivedi N, Monaghan MT, Gitomer BY, Chonchol MB, Clambey ET, Nemenoff RA, Klawitter J, Hopp K. The tryptophan-metabolizing enzyme indoleamine 2,3-dioxygenase 1 regulates polycystic kidney disease progression. JCI Insight. 2023;8(1):e154773. https://doi.org/10.1172/jci.insight.154773.

Li Z, Zimmerman KA, Cherakara S, Chumley PH, Collawn JF, Wang J, Haycraft CJ, Song CJ, Chacana T, Andersen RS, Croyle MJ, Aloria EJ, Hombal RP, Thomas IN, Chweih H, Simanyi KL, George JF, Parant JM, Mrug M, Yoder BK. A kidney resident macrophage subset is a candidate biomarker for renal cystic disease in preclinical models. Dis Model Mech. 2023 Jan 1;16(1):dmm049810. doi: 10.1242/dmm.049810. Epub 2023 Jan 16.PMID: 36457161 

Doss MC, Mullen S, Roye R, Zhou J, Chumley P, Mrug E, Wallace DP, Qian F, Harris PC, Yoder BK, Kim H, Mrug M. Accuracy and processing time of kidney volume measurement methods in rodents polycystic kidney disease models: superiority of semiautomated kidney segmentation. Am J Physiol Renal Physiol. 2023 Apr 1;324(4):F423-F430. doi: 10.1152/ajprenal.00295.2022. Epub 2023 Feb 16. PMID: 36794756

Lakhia R, Mishra A, Biggers L, Malladi V, Cobo-Stark P, Hajarnis S, Patel V. Enhancer and super-enhancer landscape in polycystic kidney disease. Kidney Int. 2023 Jan;103(1):87-99. doi: 10.1016/j.kint.2022.08.039. Epub 2022 Oct 22. PMID: 36283570