2024

Gauthier MM, Hayoz S, Banek CT. Neuroimmune interplay in kidney health and disease: Role of renal nerves. Auton Neurosci. 2023 Dec;250:103133. doi: 10.1016/j.autneu.2023.103133. Epub 2023 Nov 30. PMID: 38061177

Zhou JX, Cheng AS, Chen L, Li LX, Agborbesong E, Torres VE, Harris PC, Li X. CD74 Promotes Cyst Growth and Renal Fibrosis in Autosomal Dominant Polycystic Kidney Disease. Cells. 2024 Mar 11;13(6):489. doi: 10.3390/cells13060489. PMID: 38534333

Schönauer R, Sierks D, Boerrigter M, Jawaid T, Caroff L, Audrezet MP, Friedrich A, Shaw M, Degenhardt J, Forberger M, de Fallois J, Bläker H, Bergmann C, Gödiker J, Schindler P, Schlevogt B, Müller RU, Berg T, Patterson I, Griffiths WJ, Sayer JA; Genomics England Research Consortium; Popp B, Torres VE, Hogan MC, Somlo S, Watnick TJ, Nevens F, Besse W, Cornec-Le Gall E, Harris PC, Drenth JPH, Halbritter J. Sex, Genotype, and Liver Volume Progression as Risk of Hospitalization Determinants in Autosomal Dominant Polycystic Liver Disease. Gastroenterology 2024 166: 902-24, 2024. PMID: 38101549

Tsilosani, A., Gao, C., Chen, E., Lightle, A. R., Shehzad, S., Sharma, M., Tran, P. V., Bates, C. M., Wallace, D. P., & Zhang, W. (2024). Pkd2 Deficiency in Embryonic Aqp2 + Progenitor Cells Is Sufficient to Cause Severe Polycystic Kidney Disease. Journal of the American Society of Nephrology: JASN, 10.1681/ASN.0000000000000309. Advance online publication.

Patel, M. M., Gerakopoulos, V., Petsouki, E., Zimmerman, K. A., & Tsiokas, L. (2024). Nephronophthisis- associated FBW7 mediates cyst-dependent decline of renal function in ADPKD. bioRxiv: the preprint server for biology, 2024.02.29.582788. https://doi.org/10.1101/2024.02.29.582788

Cheng T, Mariappan A, Langner E, Shim K, Gopalakrishnan J, Mahjoub MR. Inhibiting centrosome clustering reduces cystogenesis and improves kidney function in autosomal dominant polycystic kidney disease. JCI Insight. 2024 Feb 22;9(4):e172047. doi: 10.1172/jci.insight.172047. PMID: 38385746

Mrug M, Mrug E, Rosenblum F, Chen J, Cui X, Agarwal A, Zarjou A. Distinct developmental reprogramming footprint of macrophages during acute kidney injury across species. Am J Physiol Renal Physiol. 2024;326(4):F635-F41. Epub 2024/02/15. doi: 10.1152/ajprenal.00013.2024. PubMed PMID: 38357719.

Krishnan C, Schmidt E, Onuoha E, Mrug M, Cardenas CE, Kim H. nnUNet for Automatic Kidney and Cyst Segmentation in Autosomal Dominant Polycystic Kidney Disease. Curr Med Imaging. 2024;20:1-9. Epub 2024/02/23. doi: 10.2174/0115734056272767231130110017. PubMed PMID: 38389364.

Gallini JW, Jasien CL, Mrug M, Cui X. US Veterans Administration Autosomal Dominant Polycystic Kidney Disease Cohort: Demographic, Comorbidity, and Key Laboratory Data Characteristics. Kidney360. 2024;5(4):529-37. Epub 2024/03/01. doi: 10.34067/KID.0000000000000405. PubMed PMID: 38424672.

Rezi CK, Aslanyan MG, Diwan GD, Cheng T, Chamlali M, Junger K, Anvarian Z, Lorentzen E, Pauly KB, Afshar-Bahadori Y, Fernandes EF, Qian F, Tosi S, Christensen ST, Pedersen SF, Strømgaard K, Russell RB, Miner JH, Mahjoub MR, Boldt K, Roepman R, Pedersen LB. DLG1 functions upstream of SDCCAG3 and IFT20 to control ciliary targeting of polycystin-2. EMBO Rep. 2024 Jul;25(7):3040-3063. doi: 10.1038/s44319-024-00170-1. Epub 2024 Jun 7. PMID: 38849673.

Jansson KP, Kuluva J, Zhang S, Swanson T, Zhang Y, Zimmerman KA, Fields TA, Wallace DP, Rowe PS, Stubbs JR. Osteopontin deletion attenuates cyst growth but exacerbates fibrosis in mice with cystic kidney disease. Physiol Rep. 2024 Sep;12(17):e70038. doi: 10.14814/phy2.70038. PMID: 39238069. 

Zhu M, Wang Y, Lu T, Guo J, Li L, Hsieh MH, Gopal P, Han Y, Fujiwara N, Wallace DP, Yu ASL, Fang X, Ransom C, Verschleisser S, Hsiehchen D, Hoshida Y, Singal AG, Yopp A, Wang T, Zhu H. PKD1 mutant clones within cirrhotic livers inhibit steatohepatitis without promoting cancer. Cell Metab. 2024 Aug 6;36(8):1711-1725.e8. doi: 10.1016/j.cmet.2024.05.015. PMID: 38901424.

Shetty A, Atalla A, Diggs C, Watnick T, Seliger S. Characterizing the impact of the Covid-19 pandemic on adults with autosomal dominant polycystic kidney disease: a cross-sectional study. BMC Nephrol. 2024 Aug 23;25(1):269. doi: 10.1186/s12882-024-03685-w.PMID: 39179958.

Muto Y, Yoshimura Y, Wu H, Chang-Panesso M, Ledru N, Woodward OM, Outeda P, Cheng T, Mahjoub MR, Watnick TJ, Humphreys BD. Multi-omics profiling of mouse polycystic kidney disease progression at a single cell resolution. bioRxiv [Preprint]. 2024 May 31:2024.05.27.595830. doi: 10.1101/2024.05.27.595830.PMID: 38854144.

Zuo X, Winkler B, Lerner K, Ilatovskaya DV, Zamaro AS, Dang Y, Su Y, Deng P, Fitzgibbon W, Hartman J, Park KM, Lipschutz JH. Cilia-deficient renal tubule cells are primed for injury with mitochondrial defects and aberrant tryptophan metabolism. m J Physiol Renal Physiol. 2024 Jul 1;327(1):F61-F76. doi: 10.1152/ajprenal.00225.2023. Epub 2024 May 9. PMID: 38721661.

Clerici S, Podrini C, Stefanoni D, Distefano G, Cassina L, Steidl ME, Tronci L, Canu T, Chiaravalli M, Spies D, Bell TA 3rd, Costa AS, Esposito A, D’Alessandro A, Frezza C, Bachi A, Boletta A. Inhibition of asparagine synthetase effectively retards polycystic kidney disease progression. EMBO Mol Med. 2024 Jun;16(6):1379-1403. doi: 10.1038/s44321-024-00071-9. Epub 2024 Apr 29. PMID: 38684863.

Jung HJ, Pham TD, Su XT, Grigore TV, Hoenderop JG, Olauson H, Wall SM, Ellison DH, Welling PA, Al-Qusairi L. Klotho is highly expressed in the chief sites of regulated potassium secretion, and it is stimulated by potassium intake. Sci Rep. 2024 May 10;14(1):10740. doi: 10.1038/s41598-024-61481-w.PMID: 38729987.

Bin S, Yoo M, Molinari P, Gentile M, Budge K, Cantarelli C, Khan Y, La Manna G, Baldwin WM, Dvorina N, Cravedi P, Gusella GL. Reduced decay-accelerating factor expression promotes complement-mediated cystogenesis in murine ADPKD. JCI Insight. 2024 May 23;9(12):e175220. doi: 10.1172/jci.insight.175220. PMID: 38912583.

Delgado-Rodriguez P, Lamanna-Rama N, Saande C, Aldabe R, Soto-Montenegro ML, Munoz-Barrutia A. Multiscale and multimodal evaluation of autosomal dominant polycystic kidney disease development. Commun Biol. 2024 Sep 19;7(1):1183. doi: 10.1038/s42003-024-06868-1. PMID: 39300231.

Hendrickson T, Abigail Giese A, Fiedler M, Perez W, Reyes-Sanchez E, Reyes-Lozano M, Wang S, Venegas-Zamora L, Provasek V, El-Essawi A, Breitenbach I, Fakuade FE, Kutschka I, Schiattarella GG, Voigt N, Valderrabano M, Altamirano F. Polycystin-1 loss of function increases susceptibility to atrial fibrillation through impaired DNA damage response. bioRxiv [Preprint]. 2024 Jul 11:2024.07.08.602618. doi: 10.1101/2024.07.08.602618. PMID: 39026710.

Robichaud JH, Zhang Y, Chen C, He K, Huang Y, Zhang X, Sun X, Ma X, Hardyman G, Morrison CG, Dong Z, LeBrasseur NK, Ling K, Hu J. Transiently formed nucleus-to-cilium microtubule arrays mediate senescence initiation in a KIFC3-dependent manner. Nat Commun. 2024 Sep 12;15(1):7977. doi: 10.1038/s41467-024-52363-w. PMID: 39266565.

2023

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.

Onuchic L, Padovano V, Schena G, Rajendran V, Dong K, Shi X, Pandya R, Rai V, Gresko NP, Ahmed O, Lam TT, Wang W, Shen H, Somlo S, Caplan MJ.  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.

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 .

Zimmerman KA, Song CJ, Aloria EJG, Li Z, Zhou J, Bland SJ, Yashchenko A, Crossman DK, Mrug M, Yoder BK. Early infiltrating macrophage subtype correlates with late-stage phenotypic outcome in a mouse model of hepatorenal fibrocystic disease. Lab Invest. 2021 Oct;101(10):1382-1393. doi: 10.1038/s41374-021-00627-0. PMID: 36775437.

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.

Dwivedi N, Jamadar A, Mathew S, Fields TA, Rao R. Myofibroblast depletion reduces kidney cyst growth and fibrosis in autosomal dominant polycystic kidney disease. Kidney Int. 2023 Jan;103(1):144-155. PMID: 36273656.

Houske EA, Glimm MG, Bergstrom AR, Slipher SK, Welhaven HD, Greenwood MC, Linse GM, June RK, Yu ASL, Wallace DP, Hahn AK. Metabolomic Profiling to Identify Early Urinary Biomarkers and Metabolic Pathway Alterations in Autosomal Dominant Polycystic Kidney Disease.  Am J Physiol Renal Physiol. 2023 May 4. PMID: 37141147.

Kleczko EK, Nguyen DT, Marsh KH, Bauer CD, Li AS, Monaghan M-T L, Berger MD, Furgeson SB, Gitomer BY, Chonchol MB, Clambey ET, Zimmerman KA, Nemenoff RA, Hopp K.  Immune checkpoint activity regulates polycystic kidney disease progression.  CI Insight. 2023;8(12):e161318. https://doi.org/10.1172/jci.insight.161318.

Walker RV, Yao Q, Xu H, Maranto A, Swaney KF, Ramachandran S, Li R, Cassina L, Polster BM, Outeda P, Boletta A, Watnick T, Qian F. Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and suppresses cystogenesis. Nat Commun. 2023 Oct 16;14(1):6513. doi: 10.1038/s41467-023-42196-4. PMID: 37845212.

Jung HJ, Dixon EE, Coleman R, Watnick T, Reiter JF, Outeda P, Cebotaru V, Woodward OM, Welling PA. Polycystin-2-dependent transcriptome reveals early response of autosomal dominant polycystic kidney disease. Physiol Genomics. 2023 Nov 1;55(11):565-577.

Yashchenko A, Bland SJ, Song CJ, Ahmed UKB, Sharp R, Darby IG, Cordova AM, Smith ME, Lever JM, Li Z, Aloria EJ, Khan S, Maryam B, Liu S, Crowley MR, Jones KL, Zenewicz LA, George JF, Mrug M, Crossman DK, Hopp K, Stavrakis S, Humphrey MB, Ginhoux F, Zimmerman KA. Cx3cr1 controls kidney resident macrophage heterogeneity. Front Immunol. 2023 May 15;14:1082078. doi: 10.3389/fimmu.2023.1082078. eCollection 2023. PMID: 37256130.

Akakpo JY, Ramachandran A, Rumack BH, Wallace DP, Jaeschke H. Lack of mitochondrial Cyp2E1 drives acetaminophen-induced ER stress-mediated apoptosis in mouse and human kidneys: Inhibition by 4-methylpyrazole but not N-acetylcysteine. Toxicology. 2023 Dec;500:153692. doi: 10.1016/j.tox.2023.153692. Epub 2023 Nov 30. PMID: 38042273.

Lea WA, Winklhofer T, Zelenchuk L, Sharma M, Rossol-Allison J, Fields TA, Reif G, Calvet JP, Bakeberg JL, Wallace DP, Ward CJ. Polycystin-1 Interacting Protein-1 (CU062) Interacts with the Ectodomain of Polycystin-1 (PC1). Cells. 2023 Aug 29;12(17):2166. doi: 10.3390/cells12172166. PMID: 37681898.

Wilk EJ, Howton TC, Fisher JL, Oza VH, Brownlee RT, McPherson KC, Cleary HL, Yoder BK, George JF, Mrug M, Lasseigne BN. Prioritized polycystic kidney disease drug targets and repurposing candidates from pre-cystic and cystic mouse Pkd2 model gene expression reversion. Mol Med. 2023 May 22;29(1):67. doi: 10.1186/s10020-023-00664-z. PMID: 37217845.

Yanda MK, Ciobanu C, Guggino WB, Cebotaru L. CFTR and PC2, partners in the primary cilia in autosomal dominant polycystic kidney disease. Am J Physiol Cell Physiol. 2023 Sep 1;325(3):C682-C693. doi: 10.1152/ajpcell.00197.2023.

Yanda MK, Zeidan A, Cebotaru L. Ameliorating liver disease in an autosomal recessive polycystic kidney disease mouse model. Am J Physiol Gastrointest Liver Physiol. 2023 May 1;324(5):G404-G414. doi: 10.1152/ajpgi.00255.2022. PMID: 36880660.

Dennis MR, Pires PW, Banek CT. Vascular Dysfunction in Polycystic Kidney Disease: A Mini-Review. J Vasc Res. 2023;60(3):125-136. doi: 10.1159/000531647. PMID: 37536302. 

Yu W, Wang G, Li LX, Zhang H, Gui X, Zhou JX, Calvet JP, Li X. Transcription factor FoxM1 promotes cyst growth in PKD1 mutant ADPKD. Hum Mol Genet. 2023 Mar 20;32(7):1114-1126. doi: 10.1093/hmg/ddac273.PMID: 36322156.

Lin CC, Menezes LF, Qiu J, Pearson E, Zhou F, Ishimoto Y, Anderson DE, Germino GG. In vivo Polycystin-1 interactome using a novel Pkd1 knock-in mouse model. PLoS One. 2023 Aug 4;18(8):e0289778. doi: 10.1371/journal.pone.0289778. eCollection 2023.PMID: 37540694

Ishimoto Y, Menezes LF, Zhou F, Yoshida T, Komori T, Qiu J, Young MF, Lu H, Potapova S, Outeda P, Watnick T, Germino GG. A novel ARPKD mouse model with near-complete deletion of the Polycystic Kidney and Hepatic Disease 1 (Pkhd1) genomic locus presents with multiple phenotypes but not renal cysts. Kidney Int 2023 104: 611-616, 2023.

Smith AO, Frantz WT, Preval KM, Edwards YJK, Ceol CJ, Jonassen JA, Pazour GJ. The Tumor-Associated Calcium Signal Transducer 2 (TACSTD2) oncogene is upregulated in pre-cystic epithelial cells revealing a new target for polycystic kidney disease. medRxiv [Preprint]. 2023 Dec 4:2023.12.04.23299387. doi: 10.1101/2023.12.04.23299387.PMID: 38106222

Ha K, Loeb GB, Park M, Pinedo A, Park CH, Brandes N, Ritu F, Ye CJ, Reiter JF, Delling M. ADPKD-Causing Missense Variants in Polycystin-1 Disrupt Cell Surface Localization or Polycystin Channel Function. bioRxiv [Preprint]. 2023 Dec 7:2023.12.04.570035. doi: 10.1101/2023.12.04.570035. PMID: 38106161

Rezi, CK, Aslanyan MG, Diwan GD, Cheng T, Chamlali M, Junger K, Anvarian Z, Lorentzen E, Pauly KB, Afshar-Bahadori A, Fernandes E, Qian F, Tosi S, Christensen ST, Pedersen SF, Stromgaard K, Russell RB, Miner JH, Mahjoub MR, Boldt K, Roepman R, Pedersen LB. DLG1 functions upstream of SDCCAG3 and IFT20 to control ciliary targeting of polycystin-2. bioRxiv [Preprint]. 2024 Mar 14:2023.11.10.566524. doi: 10.1101/2023.11.10.566524.

Daniel, E. A., Sommer, N. A., & Sharma, M. (2023). Polycystic kidneys: interaction of notch and renin. Clinical science (London, England: 1979), 137(15), 1145–1150.

Akakpo, J. Y., Ramachandran, A., Rumack, B. H., Wallace, D. P., & Jaeschke, H. (2023). Lack of mitochondrial Cyp2E1 drives acetaminophen-induced ER stress-mediated apoptosis in mouse and human kidneys: Inhibition by 4-methylpyrazole but not N-acetylcysteine. Toxicology, 500, 153692.

Gregory, A. V., Chebib, F. T., Poudyal, B., Holmes, H. L., Yu, A. S. L., Landsittel, D. P., Bae, K. T., Chapman, A. B., Frederic, R. O., Mrug, M., Bennett, W. M., Harris, P. C., Erickson, B. J., Torres, V. E., & Kline, T. L. (2023). Utility of new image-derived biomarkers for autosomal dominant polycystic kidney disease prognosis using automated instance cyst segmentation. Kidney international104(2), 334-342. https://doi.org/10.1016/j.kint.2023.01.010

Harafuji N, Yang C, Wu M, Thiruvengadam G, Gordish-Dressman H, Thompson RG, Bell PD, Rosenberg AZ, Dafinger C, Liebau MC, Bebok Z, Caldovic L, Guay-Woodford LM. Differential regulation of MYC expression by PKHD1/Pkhd1 in human and mouse kidneys: phenotypic implications for recessive polycystic kidney disease. Front Cell Dev Biol. 2023 Nov 17;11:1270980. doi: 10.3389/fcell.2023.1270980. eCollection 2023. PMID: 38125876.

Yang C, Harafuji N, Caldovic L, Yu W, Boddu R, Bhattacharya S, Barseghyan H, Gordish-Dressman H, Foreman O, Bebok Z, Eicher EM, Guay-Woodford LM. Pkhd1cyli/cyli mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease. J Mol Med (Berl). 2023 Sep;101(9):1141-1151. doi: 10.1007/s00109-023-02351-2. Epub 2023 Aug 16. PMID: 37584738.

Yottasan P, Chu T, Chhetri PD, Cil O. Repurposing calcium-sensing receptor activator drug cinacalcet for ADPKD treatment. Transl Res. 2024 Mar;265:17-25. doi: 10.1016/j.trsl.2023.10.005. Epub 2023 Oct 30. PMID: 37990828. 

Zhang YJ, Yang C, Wang W, Harafuji N, Stasiak P, Bell PD, Caldovic L, Sztul E, Guay-Woodford LM, Bebok Z. 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. FASEB J. 2023 Jul;37(7):e23008. doi: 10.1096/fj.202300100R. PMID: 37318790.

Claus LR, Chen C, Stallworth J, Turner JL, Slaats GG, Hawks AL, Mabillard H, Senum SR, Srikanth S, Flanagan-Steet H, Louie RJ, Silver J, Lerner-Ellis J, Morel C, Mighton C, Sleutels F, van Slegtenhorst M, van Ham T, Brooks AS, Dorresteijn EM, Barakat TS, Dahan K, Demoulin N, Goffin EJ, Olinger E; Genomics England Research Consortium; Larsen M, Hertz JM, Lilien MR, Obeidová L, Seeman T, Stone HK, Kerecuk L, Gurgu M, Yousef Yengej FA, Ammerlaan CME, Rookmaaker MB, Hanna C, Rogers RC, Duran K, Peters E, Sayer JA, van Haaften G, Harris PC, Ling K, Mason JM, van Eerde AM, Steet R. Certain heterozygous variants in the kinase domain of the serine/threonine kinase NEK8 can cause an autosomal dominant form of polycystic kidney disease. Kidney Int. 2023 Nov;104(5):995-1007. doi: 10.1016/j.kint.2023.07.021. Epub 2023 Aug 19. PMID: 37598857.

Gauthier MM, Almarabeh S, Banek CT.  A song of AAs and fire: divergent sex-dependent renal inflammatory mechanisms in hypertensive SLE mice. Am J Physiol Heart Circ Physiol. 2023 Jan 1;324(1):H82-H84. doi: 10.1152/ajpheart.00679.2022. Epub 2022 Dec 9. PMID: 36487187.

Ma X, Zhang Y, Zhang Y, Zhang X, Huang Y, He K, Chen C, Hao J, Zhao D, LeBrasseur NK, Kirkland JL, Chini EN, Wei Q, Ling K, Hu J. A stress-induced cilium-to-PML-NB route drives senescence initiation.  Nat Commun. 2023 Apr 3;14(1):1840. doi: 10.1038/s41467-023-37362-7. PMID: 37019904.

2022

Choudhury MI, Li Y, Mistriotis P, Vasconcelos ACN, Dixon EE, Yang J, Benson M, Maity D, Walker R, Martin L, Koroma F, Qian F, Konstantopoulos K, Woodward OM, Sun SX. Kidney epithelial cells are active mechano-biological fluid pumps. Nat Commun. 2022 Apr 28;13(1):2317. doi: 10.1038/s41467-022-29988-w. PMID: 35484146.

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.

Amaral AG, da Silva CCC, Serna JDC, Honorato-Sampaio K, Freitas JA, Duarte-Neto AN, Bloise AC, Cassina L, Yoshinaga MY, Chaves-Filho AB, Qian F, Miyamoto S, Boletta A, Bordin S, Kowaltowski AJ, Onuchic LF. 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.

Yanda MK, Tomar V, Cole R, Guggino WB, Cebotaru L. The Mitochondrial Ca2+ import complex is altered in ADPKD. Cell Calcium. 2022 Jan;101:102501. 34823104.

Cordido A, Vizoso-Gonzalez M, Nuñez-Gonzalez L, Molares-Vila A, Chantada-Vazquez MDP, Bravo SB, Garcia-Gonzalez MA. Quantitative Proteomic Study Unmasks Fibrinogen Pathway in Polycystic Liver Disease. Biomedicines 10:290, 2022 doi: 10.3390/biomedicines10020290. PMID: 35203500 PMCID: PMC8869147

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.

Song CJ, Li Z, Ahmed UKB, Bland SJ, Yashchenko A, Liu S, Aloria EJ, Lever JM, Gonzalez NM, Bickel MA, Giles CB, Georgescu C, Wren JD, Lang ML, Benveniste EN, Harrington LE, Tsiokas L, George JF, Jones KL, Crossman DK, Agarwal A, Mrug M, Yoder BK, Hopp K, Zimmerman KA. 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 Nephrol. 2022 Apr;33(4):747-768. doi: 10.1681/ASN.2021030278. Epub 2022 Feb 2. PMID: 35110364.

Lakhia R, Ramalingam H, Chang CM, Cobo-Stark P, Biggers L, Flaten A, Alvarez J, Valencia T, Wallace DP, Lee EC, Patel V. 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, Wu H, Omachi K, Ledru N, Wilson PC, King AJ, Eric Olson N, Gunawan MG, Kuo JJ, Cox JH, Miner JH, Seliger SL, Woodward OM, Welling PA, Watnick TJ, Humphreys BD. 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, Hwang SH, Mukhopadhyay S, Qian F. 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, Williams JC Jr, Stubbs JR, Rowe PSN, Parnell SC. 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, Maser RL, Miao Y. 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, Dai Y, Reif GA, Wallace DP.  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, Reif GA, Wallace DP, Georg GI, Jakkaraj SR, Tash JS, Yu ASL, Li X, Calvet JP.  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, Kavanaugh MA, Pottorf TS, Allard BA, Jacobs DT, Dong R, Cornelius JT, Chaturvedi A, Swenson-Fields KI, Fields TA, Pritchard MT, Sharma M, Slawson C, Wallace DP, Calvet JP, Tran PV. Ttc21b deficiency attenuates autosomal dominant polycystic kidney disease in a kidney tubular- and maturation-dependent manner. Kidney Int. 102:577-591, 2022. PMID: 35644283.

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.

Hallows KR, Abebe KZ,  Li H, Saitta B, Althouse AD, Bae KT, Lalama CM, Miskulin DC, Perrone RD, Seliger SL, Watnick TJ. Association of Longitudinal Urinary Metabolic Biomarkers With ADPKD Severity and Response to Metformin in TAME-PKD Clinical Trial Participants. Kidney Int Rep. 2022 Dec 5;8(3):467-477. doi: 10.1016/j.ekir.2022.11.019. eCollection 2023 Mar. PMID: 36938071.

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.

Li LX, Zhang X, Zhang H, Agborbesong E, Zhou JX, Calvet JP, Li X. Single-Cell and CellChat Resolution Identifies Collecting Duct Cell Subsets and Their Communications with Adjacent Cells in PKD Kidneys. Cells. 2022 Dec 22;12(1):45. doi: 10.3390/cells12010045. PMID: 36611841.

Hiratsuka K, Miyoshi T, Kroll KT, Gupta NR, Valerius MT, Ferrante T, Yamashita M, Lewis JA, Morizane R. Organoid-on-a-chip model of human ARPKD reveals mechanosensing pathomechanisms for drug discovery. Sci Adv. 2022 Sep 23;8(38):eabq0866. doi: 10.1126/sciadv.abq0866. Epub 2022 Sep 21. PMID: 36129975.

2021

Jung HJ, Coleman R, Woodward OM, Welling PA. Doxycycline Changes the Transcriptome Profile of mIMCD3 Renal Epithelial Cells. Front Physiol. 2021 Nov 5;12:771691. doi: 10.3389/fphys.2021.771691. PMID: 34803745; PMCID: PMC8602682.

Cordido A, Nuñez-Gonzalez L, Martinez-Moreno JM, Lamas-Gonzalez O, Rodriguez-Osorio L, Perez-Gomez MV, Martin-Sanchez D, Outeda P, Chiaravalli M, Watnick T, Boletta A, Diaz C, Carracedo A, Sanz AB, Ortiz A, Garcia-Gonzalez MA. 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; PMCID: PMC8455272.

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.

Aloria EJG, Song CJ, Li Z, Croyle MJ, Mrug M, Zimmerman KA, Yoder BK. Ly6chi Infiltrating Macrophages Promote Cyst Progression in Injured Conditional Ift88 Mice. Kidney 360 24: 989-95, 2021. PMID: 34396149.

Jamadar A, Suma SM, Mathew S, Fields TA, Wallace DP, Calvet JP, Rao R. 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, Stubbs JR, Zhang S, Wallace DP, Grantham JJ, Chapman AB, Torres VE, Harris PC, Bae KT, Landsittel DP, Rahbari-Oskoui FF, Mrug M, Bennett WM, Yu ASL. 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, Idowu JY, Wang W, Magenheimer B, Subramaniam D, Tran PV, Calvet JP, Wallace DP, Sharma M.  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, Yerrathota S, Dinh-Phan J, Magenheimer B, Subramaniam D, Tran PV, Zhu H, Bolisetty S, Calvet JP, Wallace DP, Sharma M. 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.

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.

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.

Zimmerman KA, Song CJ, Aloria EJG, Li Z, Zhou J, Bland SJ, Yashchenko A, Crossman DK, Mrug M, Yoder BK. Early infiltrating macrophage subtype correlates with late-stage phenotypic outcome in a mouse model of hepatorenal fibrocystic disease. Lab Invest. 2021 Oct;101(10):1382-1393. doi: 10.1038/s41374-021-00627-0. PMID: 36775437.

Agborbesong E, Zhou JX, Li LX, Calvet JP, Li X. Antioxidant enzyme peroxiredoxin 5 regulates cyst growth and ciliogenesis via modulating Plk1 stability. FASEB J. 2022 Jan;36(1):e22089. doi: 10.1096/fj.202101270RR. PMID: 34888938.

Cui X, Gallini JW, Jasien CL, Mrug M. Autosomal Dominant Polycystic Kidney Disease Does Not Significantly Alter Major COVID-19 Outcomes among Veterans. Kidney360. 2021;2(6):983-8. Epub 2022/04/05. doi: 10.34067/KID.0007282020. PubMed PMID: 35373094; PMCID: PMC8791380.

Bentley-Ford MR, Engle SE, Clearman KR, Haycraft CJ, Andersen RS, Croyle MJ, Rains AB, Berbari NF, Yoder BK. A mouse model of BBS identifies developmental and homeostatic effects of BBS5 mutation and identifies novel pituitary abnormalities. Hum Mol Genet. 2021;30(3-4):234-46. Epub 2021/02/10. doi: 10.1093/hmg/ddab039. PubMed PMID: 33560420; PMCID: PMC8355464.

Kalot MA, El Alayli A, Al Khatib M, Husainat N, McGreal K, Jalal DI, Yu ASL, Mustafa RA. A Computable Phenotype for Autosomal Dominant Polycystic Kidney Disease. Kidney360. 2021 November 25;2(11):1728-1733. PubMed PMID: 35372997; PubMed Central PMCID: PMC8785841; DOI: 10.34067/KID.0000852021.

Sousa MV, Amaral AG, Freitas JA, Murata GM, Watanabe EH, Balbo BE, Tavares MD, Hortegal RA, Rocon C, Souza LE, Irigoyen MC, Salemi VM, Onuchic LF. Smoking accelerates renal cystic disease and worsens cardiac phenotype in Pkd1-deficient mice. Sci Rep. 2021 Jul 14;11(1):14443. doi: 10.1038/s41598-021-93633-7. PMID: 34262092

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, Walker RV, Qian F, Schartner C, Cao E, Delling M. 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.

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. 2020 May 4;9:e56655. doi: 10.7554/eLife.56655. PMID: 32364494

El Ters M, Zhou X, Lepping RJ, Lu P, Karcher RT, Mahnken JD, Brooks WM, Winklhofer FT, Li X, Yu ASL. Biological Efficacy and Safety of Niacinamide in Patients With ADPKD. Kidney international reports. 2020 August;5(8):1271-1279. PubMed PMID: 32775826; PubMed Central PMCID: PMC7403550; DOI: 10.1016/j.ekir.2020.06.002.

Hu K. Quick, Coordinated and Authentic Reprogramming of Ribosome Biogenesis during iPSC Reprogramming. Cells. 2020;9(11). Epub 2020/11/19. doi: 10.3390/cells9112484. PubMed PMID: 33203179; PMCID: PMC7697288.

Cevallos RR, Edwards YJK, Parant JM, Yoder BK, Hu K. Human transcription factors responsive to initial reprogramming predominantly undergo legitimate reprogramming during fibroblast conversion to iPSCs. Sci Rep. 2020;10(1):19710. Epub 2020/11/14. doi: 10.1038/s41598-020-76705-y. PubMed PMID: 33184372; PMCID: PMC7661723.

Zhang Y, Dai Y, Raman A, Daniel E, Metcalf J, Reif G, Pierucci-Alves F, Wallace DP. Overexpression of TGF-β1 induces renal fibrosis and accelerates the decline in kidney function in polycystic kidney disease. American journal of physiology. Renal physiology. 2020 December 1;319(6):F1135-F1148. PubMed PMID: 33166182.