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Protection of Cystinotic Mice by Kidney-Specific Megalin Ablation Supports an Endocytosis-Based Mechanism for Nephropathic Cystinosis Progression.

Janssens V, Gaide Chevronnay HP, Marie S, Vincent MF, Van Der Smissen P, Nevo N, Vainio S, Nielsen R, Christensen EI, Jouret F, Antignac C, Pierreux CE, Courtoy PJ.

J Am Soc Nephrol. 2019 Nov;30(11):2177-2190. doi: 10.1681/ASN.2019040371. Epub 2019 Sep 23.


Site-specific O-glycosylation of members of the low-density lipoprotein receptor superfamily enhances ligand interactions.

Wang S, Mao Y, Narimatsu Y, Ye Z, Tian W, Goth CK, Lira-Navarrete E, Pedersen NB, Benito-Vicente A, Martin C, Uribe KB, Hurtado-Guerrero R, Christoffersen C, Seidah NG, Nielsen R, Christensen EI, Hansen L, Bennett EP, Vakhrushev SY, Schjoldager KT, Clausen H.

J Biol Chem. 2019 May 24;294(21):8349. doi: 10.1074/jbc.AAC119.009121. No abstract available.


Selective Ablation of Megalin in the Retinal Pigment Epithelium Results in Megaophthalmos, Macromelanosome Formation and Severe Retina Degeneration.

Storm T, Burgoyne T, Dunaief JL, Christensen EI, Futter C, Nielsen R.

Invest Ophthalmol Vis Sci. 2019 Jan 2;60(1):322-330. doi: 10.1167/iovs.18-25667.


APD-Containing Cyclolipodepsipeptides Target Mitochondrial Function in Hypoxic Cancer Cells.

Jacobsen KM, Villadsen NL, Tørring T, Nielsen CB, Salomón T, Nielsen MM, Tsakos M, Sibbersen C, Scavenius C, Nielsen R, Christensen EI, Guerra PF, Bross P, Pedersen JS, Enghild JJ, Johannsen M, Frøkiær J, Overgaard J, Horsman MR, Busk M, Poulsen TB.

Cell Chem Biol. 2018 Nov 15;25(11):1337-1349.e12. doi: 10.1016/j.chembiol.2018.07.010. Epub 2018 Aug 16.


Enzyme Replacement Therapy During Pregnancy in Fabry Patients : Review of Published Cases of Live Births and a New Case of a Severely Affected Female with Fabry Disease and Pre-eclampsia Complicating Pregnancy.

Madsen CV, Christensen EI, Nielsen R, Mogensen H, Rasmussen ÅK, Feldt-Rasmussen U.

JIMD Rep. 2019;44:93-101. doi: 10.1007/8904_2018_129. Epub 2018 Aug 17.


Uromodulin is expressed in the distal convoluted tubule, where it is critical for regulation of the sodium chloride cotransporter NCC.

Tokonami N, Takata T, Beyeler J, Ehrbar I, Yoshifuji A, Christensen EI, Loffing J, Devuyst O, Olinger EG.

Kidney Int. 2018 Oct;94(4):701-715. doi: 10.1016/j.kint.2018.04.021. Epub 2018 Jul 12.


The Authors Reply.

Weyer K, Birn H, Nielsen R, Christensen EI.

Kidney Int. 2018 Apr;93(4):1014-1015. doi: 10.1016/j.kint.2018.01.006. No abstract available.


Site-specific O-glycosylation of members of the low-density lipoprotein receptor superfamily enhances ligand interactions.

Wang S, Mao Y, Narimatsu Y, Ye Z, Tian W, Goth CK, Lira-Navarrete E, Pedersen NB, Benito-Vicente A, Martin C, Uribe KB, Hurtado-Guerrero R, Christoffersen C, Seidah NG, Nielsen R, Christensen EI, Hansen L, Bennett EP, Vakhrushev SY, Schjoldager KT, Clausen H.

J Biol Chem. 2018 May 11;293(19):7408-7422. doi: 10.1074/jbc.M117.817981. Epub 2018 Mar 20. Erratum in: J Biol Chem. 2019 May 24;294(21):8349.


Abolishment of proximal tubule albumin endocytosis does not affect plasma albumin during nephrotic syndrome in mice.

Weyer K, Andersen PK, Schmidt K, Mollet G, Antignac C, Birn H, Nielsen R, Christensen EI.

Kidney Int. 2018 Feb;93(2):335-342. doi: 10.1016/j.kint.2017.07.024. Epub 2017 Oct 14.


Cellular uptake of proMMP-2:TIMP-2 complexes by the endocytic receptor megalin/LRP-2.

Johanns M, Lemoine P, Janssens V, Grieco G, Moestrup SK, Nielsen R, Christensen EI, Courtoy PJ, Emonard H, Marbaix E, Henriet P.

Sci Rep. 2017 Jun 28;7(1):4328. doi: 10.1038/s41598-017-04648-y.


Megalin dependent urinary cystatin C excretion in ischemic kidney injury in rats.

Jensen D, Kierulf-Lassen C, Kristensen MLV, Nørregaard R, Weyer K, Nielsen R, Christensen EI, Birn H.

PLoS One. 2017 Jun 2;12(6):e0178796. doi: 10.1371/journal.pone.0178796. eCollection 2017.


Antibody-Directed Glucocorticoid Targeting to CD163 in M2-type Macrophages Attenuates Fructose-Induced Liver Inflammatory Changes.

Svendsen P, Graversen JH, Etzerodt A, Hager H, Røge R, Grønbæk H, Christensen EI, Møller HJ, Vilstrup H, Moestrup SK.

Mol Ther Methods Clin Dev. 2016 Dec 24;4:50-61. doi: 10.1016/j.omtm.2016.11.004. eCollection 2017 Mar 17.


Serum uromodulin-a marker of kidney function and renal parenchymal integrity.

Scherberich JE, Gruber R, Nockher WA, Christensen EI, Schmitt H, Herbst V, Block M, Kaden J, Schlumberger W.

Nephrol Dial Transplant. 2018 Feb 1;33(2):284-295. doi: 10.1093/ndt/gfw422.


Screening, diagnosis, and management of patients with Fabry disease: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

Schiffmann R, Hughes DA, Linthorst GE, Ortiz A, Svarstad E, Warnock DG, West ML, Wanner C; Conference Participants.

Kidney Int. 2017 Feb;91(2):284-293. doi: 10.1016/j.kint.2016.10.004. Epub 2016 Dec 18.


Untargeted Metabolomics Analysis of ABCC6-Deficient Mice Discloses an Altered Metabolic Liver Profile.

Rasmussen MR, Nielsen KL, Laursen MR, Nielsen CB, Svendsen P, Dimke H, Christensen EI, Johannsen M, Moestrup SK.

J Proteome Res. 2016 Dec 2;15(12):4591-4600. Epub 2016 Nov 3.


Megalin Is Predominantly Observed in Vesicular Structures in First and Third Trimester Cytotrophoblasts of the Human Placenta.

Storm T, Christensen EI, Christensen JN, Kjaergaard T, Uldbjerg N, Larsen A, Honoré B, Madsen M.

J Histochem Cytochem. 2016 Dec;64(12):769-784. Epub 2016 Oct 21.


Apoptotic properties of the type 1 interferon induced family of human mitochondrial membrane ISG12 proteins.

Gytz H, Hansen MF, Skovbjerg S, Kristensen AC, Hørlyck S, Jensen MB, Fredborg M, Markert LD, McMillan NA, Christensen EI, Martensen PM.

Biol Cell. 2017 Feb;109(2):94-112. doi: 10.1111/boc.201600034. Epub 2016 Oct 26.


Nephron morphometry in mice and rats using tomographic microscopy.

Letts RF, Zhai XY, Bhikha C, Grann BL, Blom NB, Thomsen JS, Rubin DM, Christensen EI, Andreasen A.

Am J Physiol Renal Physiol. 2017 Jan 1;312(1):F210-F229. doi: 10.1152/ajprenal.00207.2016. Epub 2016 Oct 5.


Tubular proteinuria in patients with HNF1α mutations: HNF1α drives endocytosis in the proximal tubule.

Terryn S, Tanaka K, Lengelé JP, Olinger E, Dubois-Laforgue D, Garbay S, Kozyraki R, Van Der Smissen P, Christensen EI, Courtoy PJ, Bellanné-Chantelot C, Timsit J, Pontoglio M, Devuyst O.

Kidney Int. 2016 May;89(5):1075-1089. doi: 10.1016/j.kint.2016.01.027. Epub 2016 Mar 29.


Megalin and cubilin in proximal tubule protein reabsorption: from experimental models to human disease.

Nielsen R, Christensen EI, Birn H.

Kidney Int. 2016 Jan;89(1):58-67. doi: 10.1016/j.kint.2015.11.007. Review.


Vitamin D in Tear Fluid.

Lu X, Elizondo RA, Nielsen R, Christensen EI, Yang J, Hammock BD, Watsky MA.

Invest Ophthalmol Vis Sci. 2015 Sep;56(10):5880-7. doi: 10.1167/iovs.15-17177.


Towards Automated Three-Dimensional Tracking of Nephrons through Stacked Histological Image Sets.

Bhikha C, Andreasen A, Christensen EI, Letts RF, Pantanowitz A, Rubin DM, Thomsen JS, Zhai XY.

Comput Math Methods Med. 2015;2015:545809. doi: 10.1155/2015/545809. Epub 2015 Jun 15.


In-depth phenotyping of a Donnai-Barrow patient helps clarify proximal tubule dysfunction.

Dachy A, Paquot F, Debray G, Bovy C, Christensen EI, Collard L, Jouret F.

Pediatr Nephrol. 2015 Jun;30(6):1027-31. doi: 10.1007/s00467-014-3037-7. Epub 2015 Mar 31.


Non-rigid landmark-based large-scale image registration in 3-D reconstruction of mouse and rat kidney nephrons.

Zhang YL, Chang SJ, Zhai XY, Thomsen JS, Christensen EI, Andreasen A.

Micron. 2015 Jan;68:122-129. doi: 10.1016/j.micron.2014.10.002. Epub 2014 Oct 17.


Cellular disposal of miR23b by RAB27-dependent exosome release is linked to acquisition of metastatic properties.

Ostenfeld MS, Jeppesen DK, Laurberg JR, Boysen AT, Bramsen JB, Primdal-Bengtson B, Hendrix A, Lamy P, Dagnaes-Hansen F, Rasmussen MH, Bui KH, Fristrup N, Christensen EI, Nordentoft I, Morth JP, Jensen JB, Pedersen JS, Beck M, Theodorescu D, Borre M, Howard KA, Dyrskjøt L, Ørntoft TF.

Cancer Res. 2014 Oct 15;74(20):5758-71. doi: 10.1158/0008-5472.CAN-13-3512. Epub 2014 Sep 26.


Mitochondrial localization of the OAS1 p46 isoform associated with a common single nucleotide polymorphism.

Kjær KH, Pahus J, Hansen MF, Poulsen JB, Christensen EI, Justesen J, Martensen PM.

BMC Cell Biol. 2014 Sep 9;15:33. doi: 10.1186/1471-2121-15-33.


Megalin-mediated specific uptake of chitosan/siRNA nanoparticles in mouse kidney proximal tubule epithelial cells enables AQP1 gene silencing.

Gao S, Hein S, Dagnæs-Hansen F, Weyer K, Yang C, Nielsen R, Christensen EI, Fenton RA, Kjems J.

Theranostics. 2014 Aug 13;4(10):1039-51. doi: 10.7150/thno.7866. eCollection 2014.


Biodistribution and PET imaging of a novel [68Ga]-anti-CD163-antibody conjugate in rats with collagen-induced arthritis and in controls.

Eichendorff S, Svendsen P, Bender D, Keiding S, Christensen EI, Deleuran B, Moestrup SK.

Mol Imaging Biol. 2015 Feb;17(1):87-93. doi: 10.1007/s11307-014-0768-6.


Megalin-deficiency causes high myopia, retinal pigment epithelium-macromelanosomes and abnormal development of the ciliary body in mice.

Storm T, Heegaard S, Christensen EI, Nielsen R.

Cell Tissue Res. 2014 Oct;358(1):99-107. doi: 10.1007/s00441-014-1919-4. Epub 2014 Jul 1.


Three-dimensional reconstruction of the rat nephron.

Christensen EI, Grann B, Kristoffersen IB, Skriver E, Thomsen JS, Andreasen A.

Am J Physiol Renal Physiol. 2014 Mar 15;306(6):F664-71. doi: 10.1152/ajprenal.00522.2013. Epub 2014 Jan 29.


Spatial organization of the vascular bundle and the interbundle region: three-dimensional reconstruction at the inner stripe of the outer medulla in the mouse kidney.

Ren H, Gu L, Andreasen A, Thomsen JS, Cao L, Christensen EI, Zhai XY.

Am J Physiol Renal Physiol. 2014 Feb 1;306(3):F321-6. doi: 10.1152/ajprenal.00429.2013. Epub 2013 Dec 4.


Long-term enzyme replacement therapy is associated with reduced proteinuria and preserved proximal tubular function in women with Fabry disease.

Prabakaran T, Birn H, Bibby BM, Regeniter A, Sørensen SS, Feldt-Rasmussen U, Nielsen R, Christensen EI.

Nephrol Dial Transplant. 2014 Mar;29(3):619-25. doi: 10.1093/ndt/gft452. Epub 2013 Nov 8.


Megalin in acute kidney injury: foe and friend.

Mahadevappa R, Nielsen R, Christensen EI, Birn H.

Am J Physiol Renal Physiol. 2014 Jan;306(2):F147-54. doi: 10.1152/ajprenal.00378.2013. Epub 2013 Nov 6. Review.


The authors reply.

Nielsen R, Weyer K, Birn H, Christensen EI.

Kidney Int. 2013 Nov;84(5):1050-1. doi: 10.1038/ki.2013.326. No abstract available.


Detailed investigations of proximal tubular function in Imerslund-Gräsbeck syndrome.

Storm T, Zeitz C, Cases O, Amsellem S, Verroust PJ, Madsen M, Benoist JF, Passemard S, Lebon S, Jønsson IM, Emma F, Koldsø H, Hertz JM, Nielsen R, Christensen EI, Kozyraki R.

BMC Med Genet. 2013 Oct 24;14:111. doi: 10.1186/1471-2350-14-111.


Human sorCS1 binds sortilin and hampers its cellular functions.

Larsen JV, Hermey G, Sørensen ES, Prabakaran T, Christensen EI, Gliemann J, Madsen P, Petersen CM.

Biochem J. 2014 Jan 15;457(2):277-88. doi: 10.1042/BJ20130386.


Proteinuria: Tubular handling of albumin-degradation or salvation?

Christensen EI, Birn H.

Nat Rev Nephrol. 2013 Dec;9(12):700-2. doi: 10.1038/nrneph.2013.212. Epub 2013 Oct 15.


Direct physical contact between intercalated cells in the distal convoluted tubule and the afferent arteriole in mouse kidneys.

Ren H, Liu NY, Andreasen A, Thomsen JS, Cao L, Christensen EI, Zhai XY.

PLoS One. 2013 Sep 5;8(9):e70898. doi: 10.1371/journal.pone.0070898. eCollection 2013.


Renal uptake of the antiapoptotic protein survivin is mediated by megalin at the apical membrane of the proximal tubule.

Jobst-Schwan T, Knaup KX, Nielsen R, Hackenbeck T, Buettner-Herold M, Lechler P, Kroening S, Goppelt-Struebe M, Schloetzer-Schrehardt U, Fürnrohr BG, Voll RE, Amann K, Eckardt KU, Christensen EI, Wiesener MS.

Am J Physiol Renal Physiol. 2013 Sep 1;305(5):F734-44. doi: 10.1152/ajprenal.00546.2012. Epub 2013 Jul 3.


Increased lysosomal proteolysis counteracts protein accumulation in the proximal tubule during focal segmental glomerulosclerosis.

Nielsen R, Mollet G, Esquivel EL, Weyer K, Nielsen PK, Antignac C, Christensen EI.

Kidney Int. 2013 Nov;84(5):902-10. doi: 10.1038/ki.2013.218. Epub 2013 Jun 12.


SorLA controls neurotrophic activity by sorting of GDNF and its receptors GFRα1 and RET.

Glerup S, Lume M, Olsen D, Nyengaard JR, Vaegter CB, Gustafsen C, Christensen EI, Kjolby M, Hay-Schmidt A, Bender D, Madsen P, Saarma M, Nykjaer A, Petersen CM.

Cell Rep. 2013 Jan 31;3(1):186-99. doi: 10.1016/j.celrep.2012.12.011. Epub 2013 Jan 17.


From bowel to kidneys: the role of cubilin in physiology and disease.

Christensen EI, Nielsen R, Birn H.

Nephrol Dial Transplant. 2013 Feb;28(2):274-81. doi: 10.1093/ndt/gfs565. Epub 2013 Jan 4. Review.


Haploinsufficiency of the ammonia transporter Rhcg predisposes to chronic acidosis: Rhcg is critical for apical and basolateral ammonia transport in the mouse collecting duct.

Bourgeois S, Bounoure L, Christensen EI, Ramakrishnan SK, Houillier P, Devuyst O, Wagner CA.

J Biol Chem. 2013 Feb 22;288(8):5518-29. doi: 10.1074/jbc.M112.441782. Epub 2012 Dec 31.


Renal uptake of 99mTc-dimercaptosuccinic acid is dependent on normal proximal tubule receptor-mediated endocytosis.

Weyer K, Nielsen R, Petersen SV, Christensen EI, Rehling M, Birn H.

J Nucl Med. 2013 Jan;54(1):159-65. doi: 10.2967/jnumed.112.110528. Epub 2012 Dec 11.


Renal phenotypic investigations of megalin-deficient patients: novel insights into tubular proteinuria and albumin filtration.

Storm T, Tranebjærg L, Frykholm C, Birn H, Verroust PJ, Nevéus T, Sundelin B, Hertz JM, Holmström G, Ericson K, Christensen EI, Nielsen R.

Nephrol Dial Transplant. 2013 Mar;28(3):585-91. doi: 10.1093/ndt/gfs462. Epub 2012 Oct 9.


PTH-independent regulation of blood calcium concentration by the calcium-sensing receptor.

Loupy A, Ramakrishnan SK, Wootla B, Chambrey R, de la Faille R, Bourgeois S, Bruneval P, Mandet C, Christensen EI, Faure H, Cheval L, Laghmani K, Collet C, Eladari D, Dodd RH, Ruat M, Houillier P.

J Clin Invest. 2012 Sep;122(9):3355-67. doi: 10.1172/JCI57407. Epub 2012 Aug 13.


Endocytic receptors in the renal proximal tubule.

Christensen EI, Birn H, Storm T, Weyer K, Nielsen R.

Physiology (Bethesda). 2012 Aug;27(4):223-36. doi: 10.1152/physiol.00022.2012. Review.


Mannose 6-phosphate receptor and sortilin mediated endocytosis of α-galactosidase A in kidney endothelial cells.

Prabakaran T, Nielsen R, Satchell SC, Mathieson PW, Feldt-Rasmussen U, Sørensen SS, Christensen EI.

PLoS One. 2012;7(6):e39975. doi: 10.1371/journal.pone.0039975. Epub 2012 Jun 29.


Uriniferous tubule: structural and functional organization.

Christensen EI, Wagner CA, Kaissling B.

Compr Physiol. 2012 Apr;2(2):805-61. Review.


Cubilin is expressed in rat and human glomerular podocytes.

Prabakaran T, Christensen EI, Nielsen R, Verroust PJ.

Nephrol Dial Transplant. 2012 Aug;27(8):3156-9. doi: 10.1093/ndt/gfr794. Epub 2012 Feb 15.


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