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Maryland PKD Research and Translational Core Center
DepartmentsDepartment of MedicineDivisionsDivision of NephrologyMaryland PKD Research and Translational Core CenterPublications

Publications

Publication List July 2020-September 2024

These publications used reagents supported by the Maryland PKD Research Core Center:

  1. Zimmerman KA, Hopp K, Mrug M. Role of chemokines, innate and adaptive immunity. Cellular signalling. Sep 2020;73:109647. doi:10.1016/j.cellsig.2020.109647
  2. Seliger SL, Watnick T, Althouse AD, et al. Baseline Characteristics and Patient-Reported Outcomes of ADPKD Patients in the Multicenter TAME-PKD Clinical Trial. Kidney360. Dec 31 2020;1(12):1363-1372. doi:10.34067/KID.0004002020
  3. 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. Nov 9 2020;9doi:10.7554/eLife.60684
  4. MacKay CE, Floen M, Leo MD, et al. A plasma membrane-localized polycystin-1/polycystin-2 complex in endothelial cells elicits vasodilation. Elife. Mar 1 2022;11doi:10.7554/eLife.74765
  5. Jung HJ, Dixon EE, Coleman R, et al. Polycystin-2-dependent transcriptome reveals early response of autosomal dominant polycystic kidney disease. Physiol Genomics. Nov 1 2023;55(11):565-577. doi:10.1152/physiolgenomics.00040.2023
  6. Cordido A, Nunez-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. Aug 2021;32(8):1913-1932. doi:10.1681/ASN.2020071094
  7. Perrone RD, Abebe KZ, Watnick TJ, et al. Primary results of the randomized trial of metformin administration in polycystic kidney disease (TAME PKD). Kidney Int. Sep 2021;100(3):684-696. doi:10.1016/j.kint.2021.06.013
  8. Yao Q, Outeda P, Xu H, et al. Polycystin-1 dependent regulation of polycystin-2 via GRP94, a member of HSP90 family that resides in the endoplasmic reticulum. FASEB J. Oct 2021;35(10):e21865. doi:10.1096/fj.202100325RR
  9. Aloria EJG, Song CJ, Li Z, et al. Ly6c(hi) Infiltrating Macrophages Promote Cyst Progression in Injured Conditional Ift88 Mice. Kidney360. Jun 24 2021;2(6):989-995. doi:10.34067/KID.0000882021
  10. Hopp K, Catenacci VA, Dwivedi N, et al. Weight loss and cystic disease progression in autosomal dominant polycystic kidney disease. iScience. Jan 21 2022;25(1):103697. doi:10.1016/j.isci.2021.103697
  11. Chen C, Xu Q, Zhang Y, et al. Ciliopathy protein HYLS1 coordinates the biogenesis and signaling of primary cilia by activating the ciliary lipid kinase PIPKIgamma. Sci Adv. Jun 2021;7(26)doi:10.1126/sciadv.abe3401
  12. Zimmerman KA, Song CJ, Aloria EJG, et al. Early infiltrating macrophage subtype correlates with late-stage phenotypic outcome in a mouse model of hepatorenal fibrocystic disease. Lab Invest. Oct 2021;101(10):1382-1393. doi:10.1038/s41374-021-00627-0
  13. Sousa MV, Amaral AG, Freitas JA, et al. Smoking accelerates renal cystic disease and worsens cardiac phenotype in Pkd1-deficient mice. Sci Rep. Jul 14 2021;11(1):14443. doi:10.1038/s41598-021-93633-7
  14. Choudhury MI, Li Y, Mistriotis P, et al. Kidney epithelial cells are active mechano-biological fluid pumps. Nat Commun. Apr 28 2022;13(1):2317. doi:10.1038/s41467-022-29988-w
  15. Senum SR, Li YSM, Benson KA, et al. Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype. Am J Hum Genet. Jan 6 2022;109(1):136-156. doi:10.1016/j.ajhg.2021.11.016
  16. 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. Jun 1 2022;1868(6):166371. doi:10.1016/j.bbadis.2022.166371
  17. Yanda MK, Tomar V, Cole R, Guggino WB, Cebotaru L. The Mitochondrial Ca(2+) import complex is altered in ADPKD. Cell Calcium. Jan 2022;101:102501. doi:10.1016/j.ceca.2021.102501
  18. Cordido A, Vizoso-Gonzalez M, Nunez-Gonzalez L, et al. Quantitative Proteomic Study Unmasks Fibrinogen Pathway in Polycystic Liver Disease. Biomedicines. Jan 27 2022;10(2)doi:10.3390/biomedicines10020290
  19. Song CJ, Li Z, Ahmed UKB, 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 Nephrol. Apr 2022;33(4):747-768. doi:10.1681/ASN.2021030278
  20. Lakhia R, Ramalingam H, Chang CM, et al. PKD1 and PKD2 mRNA cis-inhibition drives polycystic kidney disease progression. Nat Commun. Aug 15 2022;13(1):4765. doi:10.1038/s41467-022-32543-2
  21. 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. Oct 30 2022;13(1):6497. doi:10.1038/s41467-022-34255-z
  22. 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;9:936070. doi:10.3389/fmolb.2022.936070
  23. 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. Jul 1 2022;323(1):F59-F68. doi:10.1152/ajprenal.00165.2021
  24. Hallows KR, Li H, Saitta B, et al. Beneficial effects of bempedoic acid treatment in polycystic kidney disease cells and mice. Front Mol Biosci. 2022;9:1001941. doi:10.3389/fmolb.2022.1001941
  25. Hallows KR, Abebe KZ, Li H, et al. Association of Longitudinal Urinary Metabolic Biomarkers With ADPKD Severity and Response to Metformin in TAME-PKD Clinical Trial Participants. Kidney Int Rep. Mar 2023;8(3):467-477. doi:10.1016/j.ekir.2022.11.019
  26. 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. Aug 2022;33(8):1501-1516. doi:10.1681/ASN.2022010053
  27. Chen C, Hu J, Ling K. The Role of Primary Cilia-Associated Phosphoinositide Signaling in Development. J Dev Biol. Dec 2 2022;10(4)doi:10.3390/jdb10040051
  28. Panda DK, Bai X, Zhang Y, et al. SCF-SKP2 E3 ubiquitin ligase links mTORC1/ER stress/ISR with YAP activation in murine renal cystogenesis. J Clin Invest. Dec 15 2022;132(24)doi:10.1172/JCI153943
  29. Katsianou MA, Papavassiliou KA, Gargalionis AN, et al. Polycystin-1 regulates cell proliferation and migration through AKT/mTORC2 pathway in a human craniosynostosis cell model. J Cell Mol Med. Apr 2022;26(8):2428-2437. doi:10.1111/jcmm.17266
  30. Hiratsuka K, Miyoshi T, Kroll KT, et al. Organoid-on-a-chip model of human ARPKD reveals mechanosensing pathomechanisms for drug discovery. Sci Adv. Sep 23 2022;8(38):eabq0866. doi:10.1126/sciadv.abq0866
  31. Nguyen DT, Kleczko EK, Dwivedi N, et al. The tryptophan-metabolizing enzyme indoleamine 2,3-dioxygenase 1 regulates polycystic kidney disease progression. JCI Insight. Jan 10 2023;8(1)doi:10.1172/jci.insight.154773
  32. 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. Mar 30 2023;14(1):1790. doi:10.1038/s41467-023-37449-1
  33. Li Z, Zimmerman KA, Cherakara S, et al. A kidney resident macrophage subset is a candidate biomarker for renal cystic disease in preclinical models. Dis Model Mech. Jan 1 2023;16(1)doi:10.1242/dmm.049810
  34. Walker RV, Yao Q, Xu H, et al. Fibrocystin/Polyductin releases a C-terminal fragment that translocates into mitochondria and suppresses cystogenesis. Nat Commun. Oct 16 2023;14(1):6513. doi:10.1038/s41467-023-42196-4
  35. Yashchenko A, Bland SJ, Song CJ, et al. Cx3cr1 controls kidney resident macrophage heterogeneity. Front Immunol. 2023;14:1082078. doi:10.3389/fimmu.2023.1082078
  36. Doss MC, Mullen S, Roye R, et al. 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. Apr 1 2023;324(4):F423-F430. doi:10.1152/ajprenal.00295.2022
  37. 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. Sep 1 2023;325(3):C682-C693. doi:10.1152/ajpcell.00197.2023
  38. Yanda MK, Zeidan A, Cebotaru L. Ameliorating liver disease in an autosomal recessive polycystic kidney disease mouse model. Am J Physiol Gastrointest Liver Physiol. May 1 2023;324(5):G404-G414. doi:10.1152/ajpgi.00255.2022
  39. Lin CC, Menezes LF, Qiu J, et al. In vivo Polycystin-1 interactome using a novel Pkd1 knock-in mouse model. PLoS One. 2023;18(8):e0289778. doi:10.1371/journal.pone.0289778
  40. Ishimoto Y, Menezes LF, Zhou F, et al. 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. Sep 2023;104(3):611-616. doi:10.1016/j.kint.2023.05.027
  41. Smith AO, Frantz WT, Preval KM, et al. 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. Dec 4 2023;doi:10.1101/2023.12.04.23299387
  42. Ha K, Loeb GB, Park M, et al. ADPKD-Causing Missense Variants in Polycystin-1 Disrupt Cell Surface Localization or Polycystin Channel Function. bioRxiv. Dec 7 2023;doi:10.1101/2023.12.04.570035
  43. Harafuji N, Yang C, Wu M, et al. 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;11:1270980. doi:10.3389/fcell.2023.1270980
  44. Yang C, Harafuji N, Caldovic L, et al. Pkhd1(cyli/cyli) mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease. J Mol Med (Berl). Sep 2023;101(9):1141-1151. doi:10.1007/s00109-023-02351-2
  45. Yottasan P, Chu T, Chhetri PD, Cil O. Repurposing calcium-sensing receptor activator drug cinacalcet for ADPKD treatment. Transl Res. Mar 2024;265:17-25. doi:10.1016/j.trsl.2023.10.005
  46. Zhou JX, Cheng AS, Chen L, et al. CD74 Promotes Cyst Growth and Renal Fibrosis in Autosomal Dominant Polycystic Kidney Disease. Cells. Mar 11 2024;13(6)doi:10.3390/cells13060489
  47. Schonauer R, Sierks D, Boerrigter M, et al. Sex, Genotype, and Liver Volume Progression as Risk of Hospitalization Determinants in Autosomal Dominant Polycystic Liver Disease. Gastroenterology. May 2024;166(5):902-914. doi:10.1053/j.gastro.2023.12.007
  48. 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. Feb 22 2024;9(4)doi:10.1172/jci.insight.172047
  49. Rezi CK, Aslanyan MG, Diwan GD, et al. DLG1 functions upstream of SDCCAG3 and IFT20 to control ciliary targeting of polycystin-2. EMBO Rep. Jul 2024;25(7):3040-3063. doi:10.1038/s44319-024-00170-1
  50. 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. Aug 23 2024;25(1):269. doi:10.1186/s12882-024-03685-w
  51. Clerici S, Podrini C, Stefanoni D, et al. Inhibition of asparagine synthetase effectively retards polycystic kidney disease progression. EMBO Mol Med. Jun 2024;16(6):1379-1403. doi:10.1038/s44321-024-00071-9
  52. Jung HJ, Pham TD, Su XT, et al. Klotho is highly expressed in the chief sites of regulated potassium secretion, and it is stimulated by potassium intake. Sci Rep. May 10 2024;14(1):10740. doi:10.1038/s41598-024-61481-w
  53. Bin S, Yoo M, Molinari P, et al. Reduced decay-accelerating factor expression promotes complement-mediated cystogenesis in murine ADPKD. JCI Insight. May 23 2024;9(12)doi:10.1172/jci.insight.175220
  54. 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. Sep 19 2024;7(1):1183. doi:10.1038/s42003-024-06868-1
  55. Hendrickson T, Abigail Giese A, Fiedler M, et al. Polycystin-1 loss of function increases susceptibility to atrial fibrillation through impaired DNA damage response. bioRxiv. Jul 11 2024;doi:10.1101/2024.07.08.602618
  56. Muto Y, Yoshimura Y, Wu H, et al. Multiomics profiling of mouse polycystic kidney disease progression at a single-cell resolution. Proc Natl Acad Sci U S A. Oct 22 2024;121(43):e2410830121. doi:10.1073/pnas.2410830121
  57. Ma X, Zhang Y, Zhang Y, et al. A stress-induced cilium-to-PML-NB route drives senescence initiation. Nat Commun. Apr 3 2023;14(1):1840. doi:10.1038/s41467-023-37362-7