U.S. Department of Health and Human Services

Kidney Diseases Branch

Jeffrey B. Kopp, M.D., Chief


A selection of recent and significant publications can be viewed below.

Kaimori JY, Lin CC, Outeda P, Garcia-Gonzalez MA, Menezes LF, Hartung EA, Li A, Wu G, Fujita H, Sato Y, Nakanuma Y, Yamamoto S, Ichimaru N, Takahara S, Isaka Y, Watnick T, Onuchic LF, Guay-Woodford LM, Germino GG. NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology. Sci Rep. 2017 Aug 10; 7 (2017 Aug 10; 7; 1):7733. [Full Text/Abstract]
Outeda P, Menezes L, Hartung EA, Bridges S, Zhou F, Zhu X, Xu H, Huang Q, Yao Q, Qian F, Germino GG, Watnick T. A novel model of autosomal recessive polycystic kidney questions the role of the fibrocystin C-terminus in disease mechanism. Kidney Int. 2017 Jul 17; (2017 Jul 17). [Full Text/Abstract]
Menezes LF, Lin CC, Zhou F, Germino GG. Fatty Acid Oxidation is Impaired in An Orthologous Mouse Model of Autosomal Dominant Polycystic Kidney Disease. EBioMedicine. 2016 Mar; 5 (2016 Mar; 5):183-92. [Full Text/Abstract]
Menezes LF, Germino GG. Systems biology of polycystic kidney disease: a critical review. Wiley Interdiscip Rev Syst Biol Med. 2015 Feb 2; 7 (7; 2015 Feb 2; 1):39-52. [Full Text/Abstract]
Kim H, Xu H, Yao Q, Li W, Huang Q, Outeda P, Cebotaru V, Chiaravalli M, Boletta A, Piontek K, Germino GG, Weinman EJ, Watnick T, Qian F. Ciliary membrane proteins traffic through the Golgi via a Rabep1/GGA1/Arl3-dependent mechanism. Nat Commun. 2014 Nov 18; 5 (2014 Nov 18; 5):5482. [Full Text/Abstract]
Lee YL, Santé J, Comerci CJ, Cyge B, Menezes LF, Li FQ, Germino GG, Moerner WE, Takemaru K, Stearns T. Cby1 promotes Ahi1 recruitment to a ring-shaped domain at the centriole-cilium interface and facilitates proper cilium formation and function. Mol Biol Cell. 2014 Aug 7; 25 (25; 19; 2014 Aug 7):2919-33. [Full Text/Abstract]
Gbadegesin RA, Hall G, Adeyemo A, Hanke N, Tossidou I, Burchette J, Wu G, Homstad A, Sparks MA, Gomez J, Jiang R, Alonso A, Lavin P, Conlon P, Korstanje R, Stander MC, Shamsan G, Barua M, Spurney R, Singhal PC, Kopp JB, Haller H, Howell D, Pollak MR, Shaw AS, Schiffer M, Winn MP. Mutations in the gene that encodes the F-actin binding protein anillin cause FSGS. J Am Soc Nephrol. 2014 Mar 27; 25 (25; 2014 Mar 27; 9):1991-2002. [Full Text/Abstract]
Street JM, Yuen PS, Star RA. Bioactive Exosomes: Possibilities for Diagnosis and Management of Bladder Cancer. J Urol. 2014 Aug; 192 (2; 2014 Aug; 192):297-8. [Full Text/Abstract]
Street JM, Souza AC, Alvarez-Prats A, Horino T, Hu X, Yuen PS, Star RA. Automated quantification of renal fibrosis with Sirius Red and polarization contrast microscopy. Physiol Rep. 2014 Jul 1; 2 (2014 Jul 1; 2; 7). [Full Text/Abstract]
Wang W, Qiu L, Howard A, Solis N, Li C, Wang X, Kopp JB, Levi M. Protective effects of aliskiren and valsartan in mice with diabetic nephropathy. J Renin Angiotensin Aldosterone Syst. 2014 Jul 16; 15 (2014 Jul 16; 15; 4):384-95. [Full Text/Abstract]
Chawla LS, Eggers PW, Star RA, Kimmel PL. Acute kidney injury and chronic kidney disease as interconnected syndromes. N Engl J Med. 2014 Jul 3; 371 (1; 2014 Jul 3; 371):58-66. [Full Text/Abstract]
Boddu R, Yang C, O'Connor AK, Hendrickson RC, Boone B, Cui X, Garcia-Gonzalez M, Igarashi P, Onuchic LF, Germino GG, Guay-Woodford LM. Intragenic motifs regulate the transcriptional complexity of Pkhd1/PKHD1. J Mol Med (Berl). 2014 Jul 3; 92 (92; 10; 2014 Jul 3):1045-56. [Full Text/Abstract]
Fornoni A, Merscher S, Kopp JB. Lipid biology of the podocyte--new perspectives offer new opportunities. Nat Rev Nephrol. 2014 Jul; 10 (7; 10; 2014 Jul):379-88. [Full Text/Abstract]
Starke C, Betz H, Hickmann L, Lachmann P, Neubauer B, Kopp JB, Sequeira-Lopez ML, Gomez RA, Hohenstein B, Todorov VT, Hugo CP. Renin lineage cells repopulate the glomerular mesangium after injury. J Am Soc Nephrol. 2014 Jun 5; 26 (2014 Jun 5; 26; 1):48-54. [Full Text/Abstract]
Thomson R, Genovese G, Canon C, Kovacsics D, Higgins MK, Carrington M, Winkler CA, Kopp J, Rotimi C, Adeyemo A, Doumatey A, Ayodo G, Alper SL, Pollak MR, Friedman DJ, Raper J. Evolution of the primate trypanolytic factor APOL1. Proc Natl Acad Sci U S A. 2014 May 20; 111 (20; 2014 May 20; 111):E2130-9. [Full Text/Abstract]
Kansy K, Mueller AA, Mücke T, Kopp JB, Koersgen F, Wolff KD, Zeilhofer HF, Hölzle F, Pradel W, Schneider M, Kolk A, Smeets R, Acero J, Hoffmann J, DÖSAK Collaborative Group for Microsurgical Reconstruction. Microsurgical reconstruction of the head and neck--current concepts of maxillofacial surgery in Europe. J Craniomaxillofac Surg. 2014 May 11; 42 (8; 2014 May 11; 42):1610-3. [Full Text/Abstract]
Outeda P, Huso DL, Fisher SA, Halushka MK, Kim H, Qian F, Germino GG, Watnick T. Polycystin Signaling Is Required for Directed Endothelial Cell Migration and Lymphatic Development. Cell Rep. 2014 May 8; 7 (7; 3; 2014 May 8):634-44. [Full Text/Abstract]
Dobrinskikh E, Okamura K, Kopp JB, Doctor RB, Blaine J. Human podocytes perform polarized, caveolae-dependent albumin endocytosis. Am J Physiol Renal Physiol. 2014 May 1; 306 (2014 May 1; 9; 306):F941-51. [Full Text/Abstract]
Lee H, Abe Y, Lee I, Shrivastav S, Crusan AP, Hüttemann M, Hopfer U, Felder RA, Asico LD, Armando I, Jose PA, Kopp JB. Increased mitochondrial activity in renal proximal tubule cells from young spontaneously hypertensive rats. Kidney Int. 2014 Mar; 85 (85; 2014 Mar; 3):561-9. [Full Text/Abstract]
Carson JM, Okamura K, Wakashin H, McFann K, Dobrinskikh E, Kopp JB, Blaine J. Podocytes Degrade Endocytosed Albumin Primarily in Lysosomes. PLoS One. 2014 Jun 12; 9 (6; 9; 2014 Jun 12):e99771. [Full Text/Abstract]
Liu D, Wang CJ, Judge DP, Halushka MK, Ni J, Habashi JP, Moslehi J, Bedja D, Gabrielson KL, Xu H, Qian F, Huso D, Dietz HC, Germino GG, Watnick T. A Pkd1-Fbn1 genetic interaction implicates TGF-β signaling in the pathogenesis of vascular complications in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2014 Jan; 25 (25; 2014 Jan; 1):81-91. [Full Text/Abstract]
Parsa A, Kao WH, Xie D, Astor BC, Li M, Hsu CY, Feldman HI, Parekh RS, Kusek JW, Greene TH, Fink JC, Anderson AH, Choi MJ, Wright JT Jr, Lash JP, Freedman BI, Ojo A, Winkler CA, Raj DS, Kopp JB, He J, Jensvold NG, Tao K, Lipkowitz MS, Appel LJ, AASK Study Investigators., CRIC Study Investigators. APOL1 risk variants, race, and progression of chronic kidney disease. N Engl J Med. 2013 Dec 5; 369 (23; 2013 Dec 5; 369):2183-96. [Full Text/Abstract]
Kopp JB. JC viruria and kidney disease in APOL1 risk genotype individuals: is this a clue to a gene × environment interaction?. Kidney Int. 2013 Dec; 84 (6; 2013 Dec; 84):1069-72. [Full Text/Abstract]
Huynh N, Blain D, Glaser T, Doss EL, Zein WM, Lang DM, Baker EH, Hill S, Brewer CC, Kopp JB, Bardakjian TM, Maumenee IH, Bateman BJ, Brooks BP. Systemic diagnostic testing in patients with apparently isolated uveal coloboma. Am J Ophthalmol. 2013 Dec; 156 (156; 6; 2013 Dec):1159-1168.e4. [Full Text/Abstract]
Wang L, Teng R, Di L, Rogers H, Wu H, Kopp JB, Noguchi CT. PPARα and Sirt1 mediate erythropoietin action in increasing metabolic activity and browning of white adipocytes to protect against obesity and metabolic disorders. Diabetes. 2013 Dec; 62 (2013 Dec; 12; 62):4122-31. [Full Text/Abstract]
Kruszka PS, Manoli I, Sloan JL, Kopp JB, Venditti CP. Renal growth in isolated methylmalonic acidemia. Genet Med. 2013 Dec; 15 (2013 Dec; 15; 12):990-6. [Full Text/Abstract]
Agarwal N, Iyer D, Patel SG, Sekhar RV, Phillips TM, Schubert U, Oplt T, Buras ED, Samson SL, Couturier J, Lewis DE, Rodriguez-Barradas MC, Jahoor F, Kino T, Kopp JB, Balasubramanyam A. HIV-1 Vpr induces adipose dysfunction in vivo through reciprocal effects on PPAR/GR co-regulation. Sci Transl Med. 2013 Nov 27; 5 (213; 5; 2013 Nov 27):213ra164. [Full Text/Abstract]
Abe Y, Sakairi T, Beeson C, Kopp JB. TGF-β1 stimulates mitochondrial oxidative phosphorylation and generation of reactive oxygen species in cultured mouse podocytes, mediated in part by the mTOR pathway. Am J Physiol Renal Physiol. 2013 Nov 15; 305 (2013 Nov 15; 10; 305):F1477-90. [Full Text/Abstract]
Burghardt T, Kastner J, Suleiman H, Rivera-Milla E, Stepanova N, Lottaz C, Kubitza M, Böger CA, Schmidt S, Gorski M, de Vries U, Schmidt H, Hertting I, Kopp J, Rascle A, Moser M, Heid IM, Warth R, Spang R, Wegener J, Mierke CT, Englert C, Witzgall R. LMX1B is essential for the maintenance of differentiated podocytes in adult kidneys. J Am Soc Nephrol. 2013 Nov; 24 (24; 2013 Nov; 11):1830-48. [Full Text/Abstract]
Sharma K, Karl B, Mathew AV, Gangoiti JA, Wassel CL, Saito R, Pu M, Sharma S, You YH, Wang L, Diamond-Stanic M, Lindenmeyer MT, Forsblom C, Wu W, Ix JH, Ideker T, Kopp JB, Nigam SK, Cohen CD, Groop PH, Barshop BA, Natarajan L, Nyhan WL, Naviaux RK. Metabolomics reveals signature of mitochondrial dysfunction in diabetic kidney disease. J Am Soc Nephrol. 2013 Nov; 24 (24; 2013 Nov; 11):1901-12. [Full Text/Abstract]
Gasser DL, Winkler CA, Peng M, An P, McKenzie LA, Kirk GD, Shi Y, Xie LX, Marbois BN, Clarke CF, Kopp JB. Focal Segmental Glomerulosclerosis Is Associated With a PDSS2 Haplotype and Independently, With a Decreased Content of Coenzyme Q10. Am J Physiol Renal Physiol. 2013 Oct 15; 305 (8; 2013 Oct 15; 305):F1228-38. [Full Text/Abstract]
Bonventre JV, Basile D, Liu KD, McKay D, Molitoris BA, Nath KA, Nickolas TL, Okusa MD, Palevsky PM, Schnellmann R, Rys-Sikora K, Kimmel PL, Star RA, on behalf of the Kidney Research National Dialogue (KRND). AKI: A Path Forward. Clin J Am Soc Nephrol. 2013 Sep; 8 (8; 2013 Sep; 9):1606-8. [Full Text/Abstract]
Shrivastav S, Zhang L, Okamoto K, Lee H, Lagranha C, Abe Y, Balasubramanyam A, Lopaschuk GD, Kino T, Kopp JB. HIV-1 Vpr enhances PPARβ/δ-mediated transcription, increases PDK4 expression and reduces PDC activity. Mol Endocrinol. 2013 Sep; 27 (2013 Sep; 27; 9):1564-76. [Full Text/Abstract]
Zhou H, Kajiyama H, Tsuji T, Hu X, Leelahavanichkul A, Vento S, Frank R, Kopp JB, Trachtman H, Star RA, Yuen PS. Urinary exosomal Wilms tumor-1 as a potential biomarker for podocyte injury. Am J Physiol Renal Physiol. 2013 Aug 15; 305 (4; 2013 Aug 15; 305):F553-9. [Full Text/Abstract]
Barisoni L, Nast CC, Jennette JC, Hodgin JB, Herzenberg AM, Lemley KV, Conway CM, Kopp JB, Kretzler M, Lienczewski C, Avila-Casado C, Bagnasco S, Sethi S, Tomaszewski J, Gasim AH, Hewitt SM. Digital Pathology Evaluation in the Multicenter Nephrotic Syndrome Study Network (NEPTUNE). Clin J Am Soc Nephrol. 2013 Aug; 8 (8; 2013 Aug):1449-59. [Full Text/Abstract]
Wang L, Jia Y, Rogers H, Suzuki N, Gassmann M, Wang Q, McPherron AC, Kopp JB, Yamamoto M, Noguchi CT. Erythropoietin contributes to slow oxidative muscle fiber specification via PGC-1α and AMPK activation. Int J Biochem Cell Biol. 2013 Jul; 45 (7; 45; 2013 Jul):1155-64. [Full Text/Abstract]
Kopp JB. An Expanding Universe of FSGS Genes and Phenotypes: LMX1B Mutations Cause Familial Autosomal Dominant FSGS Lacking Extrarenal Manifestations. J Am Soc Nephrol. 2013 Jul; 24 (8; 24; 2013 Jul):1183-5. [Full Text/Abstract]
Zhou H, Hasni SA, Perez P, Tandon M, Jang SI, Zheng C, Kopp JB, Austin H 3rd, Balow JE, Alevizos I, Illei GG. miR-150 promotes renal fibrosis in lupus nephritis by downregulating SOCS1. J Am Soc Nephrol. 2013 Jun; 24 (7; 24; 2013 Jun):1073-87. [Full Text/Abstract]
Lempert KD, Kopp JB. Hurricane Sandy as a kidney failure disaster. Am J Kidney Dis. 2013 Jun; 61 (6; 2013 Jun; 61):865-8. [Full Text/Abstract]
Watnick TJ, Germino GG. Polycystic kidney disease: Polycystin-1 and polycystin-2--it's complicated. Nat Rev Nephrol. 2013 May; 9 (2013 May; 9; 5):249-50. [Full Text/Abstract]
Kopp JB. Rethinking hypertensive kidney disease: arterionephrosclerosis as a genetic, metabolic, and inflammatory disorder. Curr Opin Nephrol Hypertens. 2013 May; 22 (2013 May; 3; 22):266-72. [Full Text/Abstract]
Purswani M, Patel K, Kopp JB, Seage GR 3rd, Chernoff MC, Hazra R, Siberry GK, Mofenson LM, Scott GB, Van Dyke RB, for the Pediatric HIVAIDS Cohort Study. Tenofovir Treatment Duration Predicts Proteinuria in a Multiethnic United States Cohort of Children and Adolescents With Perinatal HIV-1 Infection. Pediatr Infect Dis J. 2013 May; 32 (2013 May; 32; 5):495-500. [Full Text/Abstract]
Gadegbeku CA, Gipson DS, Holzman LB, Ojo AO, Song PX, Barisoni L, Sampson MG, Kopp JB, Lemley KV, Nelson PJ, Lienczewski CC, Adler SG, Appel GB, Cattran DC, Choi MJ, Contreras G, Dell KM, Fervenza FC, Gibson KL, Greenbaum LA, Hernandez JD, Hewitt SM, Hingorani SR, Hladunewich M, Hogan MC, Hogan SL, Kaskel FJ, Lieske JC, Meyers KE, Nachman PH, Nast CC, Neu AM, Reich HN, Sedor JR, Sethna CB, Trachtman H, Tuttle KR, Zhdanova O, Zilleruelo GE, Kretzler M. Design of the Nephrotic Syndrome Study Network (NEPTUNE) to evaluate primary glomerular nephropathy by a multidisciplinary approach. Kidney Int. 2013 Apr; 83 (83; 4; 2013 Apr):749-56. [Full Text/Abstract]
Kopp JB. Glomerular disease in 2012: more mechanistic insights, but translational progress is slow. Nat Rev Nephrol. 2013 Feb; 9 (2; 2013 Feb; 9):67-8. [Full Text/Abstract]
Chandra P, Kopp JB. Viruses and collapsing glomerulopathy: a brief critical review. Clin Kidney J. 2013 Feb; 6 (6; 2013 Feb; 1):1-5. [Full Text/Abstract]
Cravedi P, Kopp JB, Remuzzi G. Recent progress in the pathophysiology and treatment of FSGS recurrence. Am J Transplant. 2013 Feb; 13 (2; 2013 Feb; 13):266-74. [Full Text/Abstract]
Lipkowitz MS, Freedman BI, Langefeld CD, Comeau ME, Bowden DW, Kao WH, Astor BC, Bottinger EP, Iyengar SK, Klotman PE, Freedman RG, Zhang W, Parekh RS, Choi MJ, Nelson GW, Winkler CA, Kopp JB, SK Investigators. Apolipoprotein L1 gene variants associate with hypertension-attributed nephropathy and the rate of kidney function decline in African Americans. Kidney Int. 2013 Jan; 83 (83; 1; 2013 Jan):114-20. [Full Text/Abstract]
Hadigan C, Edwards E, Rosenberg A, Purdy JB, Fleischman E, Howard L, Mican JM, Sampath K, Oyalowo A, Johnson A, Adler A, Rehm C, Smith M, Lai L, Kopp JB. Microalbuminuria in HIV disease. Am J Nephrol. 2013; 37 (37; 5; 2013):443-51. [Full Text/Abstract]
Guess AJ, Ayoob R, Chanley M, Manley J, Cajaiba MM, Agrawal S, Pengal R, Pyle AL, Becknell B, Kopp JB, Ronkina N, Gaestel M, Benndorf R, Smoyer WE. Crucial roles of the protein kinases MK2 and MK3 in a mouse model of glomerulonephritis. PLoS One. 2013; 8 (8; 1; 2013):e54239. [Full Text/Abstract]
Okamura K, Dummer P, Kopp J, Qiu L, Levi M, Faubel S, Blaine J. Endocytosis of albumin by podocytes elicits an inflammatory response and induces apoptotic cell death. PLoS One. 2013; 8 (8; 1; 2013):e54817. [Full Text/Abstract]