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Lysosomal proteome analysis reveals that CLN3-defective cells have multiple enzyme deficiencies associated with changes in intracellular trafficking.

Schmidtke C, Tiede S, Thelen M, Käkelä R, Jabs S, Makrypidi G, Sylvester M, Schweizer M, Braren I, Brocke-Ahmadinejad N, Cotman SL, Schulz A, Gieselmann V, Braulke T.

J Biol Chem. 2019 Jun 14;294(24):9592-9604. doi: 10.1074/jbc.RA119.008852. Epub 2019 Apr 30.


Influenza binds phosphorylated glycans from human lung.

Byrd-Leotis L, Jia N, Dutta S, Trost JF, Gao C, Cummings SF, Braulke T, Müller-Loennies S, Heimburg-Molinaro J, Steinhauer DA, Cummings RD.

Sci Adv. 2019 Feb 13;5(2):eaav2554. doi: 10.1126/sciadv.aav2554. eCollection 2019 Feb.


Disease-Linked Glutarylation Impairs Function and Interactions of Mitochondrial Proteins and Contributes to Mitochondrial Heterogeneity.

Schmiesing J, Storch S, Dörfler AC, Schweizer M, Makrypidi-Fraune G, Thelen M, Sylvester M, Gieselmann V, Meyer-Schwesinger C, Koch-Nolte F, Tidow H, Mühlhausen C, Waheed A, Sly WS, Braulke T.

Cell Rep. 2018 Sep 11;24(11):2946-2956. doi: 10.1016/j.celrep.2018.08.014.


The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover.

Pohl S, Angermann A, Jeschke A, Hendrickx G, Yorgan TA, Makrypidi-Fraune G, Steigert A, Kuehn SC, Rolvien T, Schweizer M, Koehne T, Neven M, Winter O, Velho RV, Albers J, Streichert T, Pestka JM, Baldauf C, Breyer S, Stuecker R, Muschol N, Cox TM, Saftig P, Paganini C, Rossi A, Amling M, Braulke T, Schinke T.

J Bone Miner Res. 2018 Dec;33(12):2186-2201. doi: 10.1002/jbmr.3563. Epub 2018 Aug 24.


Lysosomal Proteome and Secretome Analysis Identifies Missorted Enzymes and Their Nondegraded Substrates in Mucolipidosis III Mouse Cells.

Di Lorenzo G, Velho RV, Winter D, Thelen M, Ahmadi S, Schweizer M, De Pace R, Cornils K, Yorgan TA, Grüb S, Hermans-Borgmeyer I, Schinke T, Müller-Loennies S, Braulke T, Pohl S.

Mol Cell Proteomics. 2018 Aug;17(8):1612-1626. doi: 10.1074/mcp.RA118.000720. Epub 2018 May 17.


GNPTAB missense mutations cause loss of GlcNAc-1-phosphotransferase activity in mucolipidosis type II through distinct mechanisms.

Ludwig NF, Velho RV, Sperb-Ludwig F, Acosta AX, Ribeiro EM, Kim CA, Gandelman Horovitz DD, Boy R, Rodovalho-Doriqui MJ, Lourenço CM, Santos ES, Braulke T, Pohl S, Schwartz IVD.

Int J Biochem Cell Biol. 2017 Nov;92:90-94. doi: 10.1016/j.biocel.2017.09.006. Epub 2017 Sep 14.


Site-1 protease and lysosomal homeostasis.

Velho RV, De Pace R, Klünder S, Di Lorenzo G, Schweizer M, Braulke T, Pohl S.

Biochim Biophys Acta Mol Cell Res. 2017 Nov;1864(11 Pt B):2162-2168. doi: 10.1016/j.bbamcr.2017.06.023. Epub 2017 Jul 8. Review.


SILAC-Based Comparative Proteomic Analysis of Lysosomes from Mammalian Cells Using LC-MS/MS.

Thelen M, Winter D, Braulke T, Gieselmann V.

Methods Mol Biol. 2017;1594:1-18. doi: 10.1007/978-1-4939-6934-0_1.


Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II.

Markmann S, Krambeck S, Hughes CJ, Mirzaian M, Aerts JM, Saftig P, Schweizer M, Vissers JP, Braulke T, Damme M.

Mol Cell Proteomics. 2017 Mar;16(3):438-450. doi: 10.1074/mcp.M116.063636. Epub 2017 Jan 6.


Disease-causing mutations affecting surface residues of mitochondrial glutaryl-CoA dehydrogenase impair stability, heteromeric complex formation and mitochondria architecture.

Schmiesing J, Lohmöller B, Schweizer M, Tidow H, Gersting SW, Muntau AC, Braulke T, Mühlhausen C.

Hum Mol Genet. 2017 Feb 1;26(3):538-551. doi: 10.1093/hmg/ddw411.


Identification of the interaction domains between α- and γ-subunits of GlcNAc-1-phosphotransferase.

Velho RV, De Pace R, Tidow H, Braulke T, Pohl S.

FEBS Lett. 2016 Dec;590(23):4287-4295. doi: 10.1002/1873-3468.12456. Epub 2016 Oct 25.


Mannose 6-phosphate-dependent targeting of lysosomal enzymes is required for normal craniofacial and dental development.

Koehne T, Markmann S, Schweizer M, Muschol N, Friedrich RE, Hagel C, Glatzel M, Kahl-Nieke B, Amling M, Schinke T, Braulke T.

Biochim Biophys Acta. 2016 Sep;1862(9):1570-80. doi: 10.1016/j.bbadis.2016.05.018. Epub 2016 May 27.


Single-chain antibody-fragment M6P-1 possesses a mannose 6-phosphate monosaccharide-specific binding pocket that distinguishes N-glycan phosphorylation in a branch-specific manner†.

Blackler RJ, Evans DW, Smith DF, Cummings RD, Brooks CL, Braulke T, Liu X, Evans SV, Müller-Loennies S.

Glycobiology. 2016 Feb;26(2):181-92. doi: 10.1093/glycob/cwv093. Epub 2015 Oct 26.


Impaired bone remodeling and its correction by combination therapy in a mouse model of mucopolysaccharidosis-I.

Kuehn SC, Koehne T, Cornils K, Markmann S, Riedel C, Pestka JM, Schweizer M, Baldauf C, Yorgan TA, Krause M, Keller J, Neven M, Breyer S, Stuecker R, Muschol N, Busse B, Braulke T, Fehse B, Amling M, Schinke T.

Hum Mol Genet. 2015 Dec 15;24(24):7075-86. doi: 10.1093/hmg/ddv407. Epub 2015 Oct 1.


Subunit interactions of the disease-related hexameric GlcNAc-1-phosphotransferase complex.

De Pace R, Velho RV, Encarnação M, Marschner K, Braulke T, Pohl S.

Hum Mol Genet. 2015 Dec 1;24(23):6826-35. doi: 10.1093/hmg/ddv387. Epub 2015 Sep 18.


In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11.

Varga RE, Khundadze M, Damme M, Nietzsche S, Hoffmann B, Stauber T, Koch N, Hennings JC, Franzka P, Huebner AK, Kessels MM, Biskup C, Jentsch TJ, Qualmann B, Braulke T, Kurth I, Beetz C, Hübner CA.

PLoS Genet. 2015 Aug 18;11(8):e1005454. doi: 10.1371/journal.pgen.1005454. eCollection 2015 Aug.


Mannose 6-phosphate-independent Lysosomal Sorting of LIMP-2.

Blanz J, Zunke F, Markmann S, Damme M, Braulke T, Saftig P, Schwake M.

Traffic. 2015 Oct;16(10):1127-36. doi: 10.1111/tra.12313. Epub 2015 Sep 1.


Site-1 protease-activated formation of lysosomal targeting motifs is independent of the lipogenic transcription control.

Klünder S, Heeren J, Markmann S, Santer R, Braulke T, Pohl S.

J Lipid Res. 2015 Aug;56(8):1625-32. doi: 10.1194/jlr.M060756. Epub 2015 Jun 24.


Lysoplex: An efficient toolkit to detect DNA sequence variations in the autophagy-lysosomal pathway.

Di Fruscio G, Schulz A, De Cegli R, Savarese M, Mutarelli M, Parenti G, Banfi S, Braulke T, Nigro V, Ballabio A.

Autophagy. 2015;11(6):928-38. doi: 10.1080/15548627.2015.1043077.


Sustained Neural Stem Cell-Based Intraocular Delivery of CNTF Attenuates Photoreceptor Loss in the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis.

Jankowiak W, Kruszewski K, Flachsbarth K, Skevas C, Richard G, Rüther K, Braulke T, Bartsch U.

PLoS One. 2015 May 20;10(5):e0127204. doi: 10.1371/journal.pone.0127204. eCollection 2015.


Analyses of disease-related GNPTAB mutations define a novel GlcNAc-1-phosphotransferase interaction domain and an alternative site-1 protease cleavage site.

Velho RV, De Pace R, Klünder S, Sperb-Ludwig F, Lourenço CM, Schwartz IV, Braulke T, Pohl S.

Hum Mol Genet. 2015 Jun 15;24(12):3497-505. doi: 10.1093/hmg/ddv100. Epub 2015 Mar 18.


Lrp1/LDL Receptor Play Critical Roles in Mannose 6-Phosphate-Independent Lysosomal Enzyme Targeting.

Markmann S, Thelen M, Cornils K, Schweizer M, Brocke-Ahmadinejad N, Willnow T, Heeren J, Gieselmann V, Braulke T, Kollmann K.

Traffic. 2015 Jul;16(7):743-59. doi: 10.1111/tra.12284. Epub 2015 Apr 27.


Mannose 6 phosphorylation of lysosomal enzymes controls B cell functions.

Otomo T, Schweizer M, Kollmann K, Schumacher V, Muschol N, Tolosa E, Mittrücker HW, Braulke T.

J Cell Biol. 2015 Jan 19;208(2):171-80. doi: 10.1083/jcb.201407077.


Molecular characterization of arylsulfatase G: expression, processing, glycosylation, transport, and activity.

Kowalewski B, Lübke T, Kollmann K, Braulke T, Reinheckel T, Dierks T, Damme M.

J Biol Chem. 2014 Oct 3;289(40):27992-8005. doi: 10.1074/jbc.M114.584144. Epub 2014 Aug 18.


Lysine glutarylation is a protein posttranslational modification regulated by SIRT5.

Tan M, Peng C, Anderson KA, Chhoy P, Xie Z, Dai L, Park J, Chen Y, Huang H, Zhang Y, Ro J, Wagner GR, Green MF, Madsen AS, Schmiesing J, Peterson BS, Xu G, Ilkayeva OR, Muehlbauer MJ, Braulke T, Mühlhausen C, Backos DS, Olsen CA, McGuire PJ, Pletcher SD, Lombard DB, Hirschey MD, Zhao Y.

Cell Metab. 2014 Apr 1;19(4):605-17. doi: 10.1016/j.cmet.2014.03.014.


Interaction of glutaric aciduria type 1-related glutaryl-CoA dehydrogenase with mitochondrial matrix proteins.

Schmiesing J, Schlüter H, Ullrich K, Braulke T, Mühlhausen C.

PLoS One. 2014 Feb 3;9(2):e87715. doi: 10.1371/journal.pone.0087715. eCollection 2014.


Mucolipidosis II-related mutations inhibit the exit from the endoplasmic reticulum and proteolytic cleavage of GlcNAc-1-phosphotransferase precursor protein (GNPTAB).

De Pace R, Coutinho MF, Koch-Nolte F, Haag F, Prata MJ, Alves S, Braulke T, Pohl S.

Hum Mutat. 2014 Mar;35(3):368-76. doi: 10.1002/humu.22502. Epub 2014 Jan 15.


A hereditary spastic paraplegia mouse model supports a role of ZFYVE26/SPASTIZIN for the endolysosomal system.

Khundadze M, Kollmann K, Koch N, Biskup C, Nietzsche S, Zimmer G, Hennings JC, Huebner AK, Symmank J, Jahic A, Ilina EI, Karle K, Schöls L, Kessels M, Braulke T, Qualmann B, Kurth I, Beetz C, Hübner CA.

PLoS Genet. 2013;9(12):e1003988. doi: 10.1371/journal.pgen.1003988. Epub 2013 Dec 19.


Decreased bone formation and increased osteoclastogenesis cause bone loss in mucolipidosis II.

Kollmann K, Pestka JM, Kühn SC, Schöne E, Schweizer M, Karkmann K, Otomo T, Catala-Lehnen P, Failla AV, Marshall RP, Krause M, Santer R, Amling M, Braulke T, Schinke T.

EMBO Mol Med. 2013 Dec;5(12):1871-86. doi: 10.1002/emmm.201302979. Epub 2013 Oct 15.


Apoptotic photoreceptor loss and altered expression of lysosomal proteins in the nclf mouse model of neuronal ceroid lipofuscinosis.

Bartsch U, Galliciotti G, Jofre GF, Jankowiak W, Hagel C, Braulke T.

Invest Ophthalmol Vis Sci. 2013 Oct 23;54(10):6952-9. doi: 10.1167/iovs.13-12945.


Ultrastructural analysis of neuronal and non-neuronal lysosomal storage in mucolipidosis type II knock-in mice.

Schweizer M, Markmann S, Braulke T, Kollmann K.

Ultrastruct Pathol. 2013 Oct;37(5):366-72. doi: 10.3109/01913123.2013.810687.


Evaluation of butyrate-induced production of a mannose-6-phosphorylated therapeutic enzyme using parallel bioreactors.

Madhavarao CN, Agarabi CD, Wong L, Müller-Loennies S, Braulke T, Khan M, Anderson H, Johnson GR.

Biotechnol Appl Biochem. 2014 Mar-Apr;61(2):184-92. doi: 10.1002/bab.1151. Epub 2014 Feb 20.


Acute renal proximal tubule alterations during induced metabolic crises in a mouse model of glutaric aciduria type 1.

Thies B, Meyer-Schwesinger C, Lamp J, Schweizer M, Koeller DM, Ullrich K, Braulke T, Mühlhausen C.

Biochim Biophys Acta. 2013 Oct;1832(10):1463-72. doi: 10.1016/j.bbadis.2013.04.019. Epub 2013 Apr 24.


Cell biology and function of neuronal ceroid lipofuscinosis-related proteins.

Kollmann K, Uusi-Rauva K, Scifo E, Tyynelä J, Jalanko A, Braulke T.

Biochim Biophys Acta. 2013 Nov;1832(11):1866-81. doi: 10.1016/j.bbadis.2013.01.019. Epub 2013 Feb 9. Review.


Mass spectrometric analysis of neutral and anionic N-glycans from a Dictyostelium discoideum model for human congenital disorder of glycosylation CDG IL.

Hykollari A, Balog CI, Rendić D, Braulke T, Wilson IB, Paschinger K.

J Proteome Res. 2013 Mar 1;12(3):1173-87. doi: 10.1021/pr300806b. Epub 2013 Jan 27.


Transport of the GlcNAc-1-phosphotransferase α/β-subunit precursor protein to the Golgi apparatus requires a combinatorial sorting motif.

Franke M, Braulke T, Storch S.

J Biol Chem. 2013 Jan 11;288(2):1238-49. doi: 10.1074/jbc.M112.407676. Epub 2012 Nov 28.


Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice.

Kollmann K, Damme M, Markmann S, Morelle W, Schweizer M, Hermans-Borgmeyer I, Röchert AK, Pohl S, Lübke T, Michalski JC, Käkelä R, Walkley SU, Braulke T.

Brain. 2012 Sep;135(Pt 9):2661-75. doi: 10.1093/brain/aws209.


The 5-phosphatase OCRL mediates retrograde transport of the mannose 6-phosphate receptor by regulating a Rac1-cofilin signalling module.

van Rahden VA, Brand K, Najm J, Heeren J, Pfeffer SR, Braulke T, Kutsche K.

Hum Mol Genet. 2012 Dec 1;21(23):5019-38. doi: 10.1093/hmg/dds343. Epub 2012 Aug 19.


Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.

Thelen M, Damme M, Schweizer M, Hagel C, Wong AM, Cooper JD, Braulke T, Galliciotti G.

PLoS One. 2012;7(4):e35493. doi: 10.1371/journal.pone.0035493. Epub 2012 Apr 20. Erratum in: PLoS One. 2012;7(5): doi/10.1371/annotation/a4b06d46-8eb9-4d15-a15a-41bf4b5ccb8b. Daμμe, Markus [corrected to Damme, Markus]..


Mannose 6 dephosphorylation of lysosomal proteins mediated by acid phosphatases Acp2 and Acp5.

Makrypidi G, Damme M, Müller-Loennies S, Trusch M, Schmidt B, Schlüter H, Heeren J, Lübke T, Saftig P, Braulke T.

Mol Cell Biol. 2012 Feb;32(4):774-82. doi: 10.1128/MCB.06195-11. Epub 2011 Dec 12.


High expression of disease-related Cln6 in the cerebral cortex, purkinje cells, dentate gyrus, and hippocampal ca1 neurons.

Thelen M, Fehr S, Schweizer M, Braulke T, Galliciotti G.

J Neurosci Res. 2012 Mar;90(3):568-74. doi: 10.1002/jnr.22773. Epub 2011 Oct 19.


N-glycans and glycosylphosphatidylinositol-anchor act on polarized sorting of mouse PrP(C) in Madin-Darby canine kidney cells.

Puig B, Altmeppen HC, Thurm D, Geissen M, Conrad C, Braulke T, Glatzel M.

PLoS One. 2011;6(9):e24624. doi: 10.1371/journal.pone.0024624. Epub 2011 Sep 8.


Analysis of potential biomarkers and modifier genes affecting the clinical course of CLN3 disease.

Lebrun AH, Moll-Khosrawi P, Pohl S, Makrypidi G, Storch S, Kilian D, Streichert T, Otto B, Mole SE, Ullrich K, Cotman S, Kohlschütter A, Braulke T, Schulz A.

Mol Med. 2011;17(11-12):1253-61. doi: 10.2119/molmed.2010.00241. Epub 2011 Aug 18.


A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism.

Marschner K, Kollmann K, Schweizer M, Braulke T, Pohl S.

Science. 2011 Jul 1;333(6038):87-90. doi: 10.1126/science.1205677.


Residual activity and proteasomal degradation of p.Ser298Pro sulfamidase identified in patients with a mild clinical phenotype of Sanfilippo A syndrome.

Muschol N, Pohl S, Meyer A, Gal A, Ullrich K, Braulke T.

Am J Med Genet A. 2011 Jul;155A(7):1634-9. doi: 10.1002/ajmg.a.34053. Epub 2011 Jun 10.


Glutaric aciduria type 1 metabolites impair the succinate transport from astrocytic to neuronal cells.

Lamp J, Keyser B, Koeller DM, Ullrich K, Braulke T, Mühlhausen C.

J Biol Chem. 2011 May 20;286(20):17777-84. doi: 10.1074/jbc.M111.232744. Epub 2011 Mar 29.


Post-translational modifications of the gamma-subunit affect intracellular trafficking and complex assembly of GlcNAc-1-phosphotransferase.

Encarnação M, Kollmann K, Trusch M, Braulke T, Pohl S.

J Biol Chem. 2011 Feb 18;286(7):5311-8. doi: 10.1074/jbc.M110.202382. Epub 2010 Dec 20.


Protein kinase a dependent phosphorylation of apical membrane antigen 1 plays an important role in erythrocyte invasion by the malaria parasite.

Leykauf K, Treeck M, Gilson PR, Nebl T, Braulke T, Cowman AF, Gilberger TW, Crabb BS.

PLoS Pathog. 2010 Jun 3;6(6):e1000941. doi: 10.1371/journal.ppat.1000941.


Proteolytic processing of the gamma-subunit is associated with the failure to form GlcNAc-1-phosphotransferase complexes and mannose 6-phosphate residues on lysosomal enzymes in human macrophages.

Pohl S, Tiede S, Marschner K, Encarnação M, Castrichini M, Kollmann K, Muschol N, Ullrich K, Müller-Loennies S, Braulke T.

J Biol Chem. 2010 Jul 30;285(31):23936-44. doi: 10.1074/jbc.M110.129684. Epub 2010 May 19.


A novel single-chain antibody fragment for detection of mannose 6-phosphate-containing proteins: application in mucolipidosis type II patients and mice.

Müller-Loennies S, Galliciotti G, Kollmann K, Glatzel M, Braulke T.

Am J Pathol. 2010 Jul;177(1):240-7. doi: 10.2353/ajpath.2010.090954. Epub 2010 May 14.

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