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Items: 1 to 50 of 378

1.

Eukaryotic formylglycine-generating enzyme catalyses a monooxygenase type of reaction.

Peng J, Alam S, Radhakrishnan K, Mariappan M, Rudolph MG, May C, Dierks T, von Figura K, Schmidt B.

FEBS J. 2015 Sep;282(17):3262-74. doi: 10.1111/febs.13347. Epub 2015 Jul 7.

2.

Differential involvement of the extracellular 6-O-endosulfatases Sulf1 and Sulf2 in brain development and neuronal and behavioural plasticity.

Kalus I, Salmen B, Viebahn C, von Figura K, Schmitz D, D'Hooge R, Dierks T.

J Cell Mol Med. 2009 Nov-Dec;13(11-12):4505-21. doi: 10.1111/j.1582-4934.2008.00558.x.

3.

Molecular basis of multiple sulfatase deficiency, mucolipidosis II/III and Niemann-Pick C1 disease - Lysosomal storage disorders caused by defects of non-lysosomal proteins.

Dierks T, Schlotawa L, Frese MA, Radhakrishnan K, von Figura K, Schmidt B.

Biochim Biophys Acta. 2009 Apr;1793(4):710-25. doi: 10.1016/j.bbamcr.2008.11.015. Epub 2008 Dec 10. Review.

4.

AP-1 and AP-3 mediate sorting of melanosomal and lysosomal membrane proteins into distinct post-Golgi trafficking pathways.

Chapuy B, Tikkanen R, Mühlhausen C, Wenzel D, von Figura K, Höning S.

Traffic. 2008 Jul;9(7):1157-72. doi: 10.1111/j.1600-0854.2008.00745.x. Epub 2008 Apr 9.

5.

The non-catalytic N-terminal extension of formylglycine-generating enzyme is required for its biological activity and retention in the endoplasmic reticulum.

Mariappan M, Gande SL, Radhakrishnan K, Schmidt B, Dierks T, von Figura K.

J Biol Chem. 2008 Apr 25;283(17):11556-64. doi: 10.1074/jbc.M707858200. Epub 2008 Feb 27.

6.

Paralog of the formylglycine-generating enzyme--retention in the endoplasmic reticulum by canonical and noncanonical signals.

Gande SL, Mariappan M, Schmidt B, Pringle TH, von Figura K, Dierks T.

FEBS J. 2008 Mar;275(6):1118-30. doi: 10.1111/j.1742-4658.2008.06271.x. Epub 2008 Feb 6.

7.

ERp44 mediates a thiol-independent retention of formylglycine-generating enzyme in the endoplasmic reticulum.

Mariappan M, Radhakrishnan K, Dierks T, Schmidt B, von Figura K.

J Biol Chem. 2008 Mar 7;283(10):6375-83. doi: 10.1074/jbc.M709171200. Epub 2008 Jan 4.

8.
9.

Structure-function relationship for lysosomal enzymes.

von Figura K.

Acta Paediatr. 2007 Apr;96(455):5. No abstract available.

PMID:
17391431
10.

Golgi GDP-fucose transporter-deficient mice mimic congenital disorder of glycosylation IIc/leukocyte adhesion deficiency II.

Hellbusch CC, Sperandio M, Frommhold D, Yakubenia S, Wild MK, Popovici D, Vestweber D, Gröne HJ, von Figura K, Lübke T, Körner C.

J Biol Chem. 2007 Apr 6;282(14):10762-72. Epub 2007 Feb 2.

11.

Molecular characterization of the hypothetical 66.3-kDa protein in mouse: lysosomal targeting, glycosylation, processing and tissue distribution.

Deuschl F, Kollmann K, von Figura K, Lübke T.

FEBS Lett. 2006 Oct 16;580(24):5747-52. Epub 2006 Sep 22.

12.

Heparan sulfate 6-O-endosulfatases: discrete in vivo activities and functional co-operativity.

Lamanna WC, Baldwin RJ, Padva M, Kalus I, Ten Dam G, van Kuppevelt TH, Gallagher JT, von Figura K, Dierks T, Merry CL.

Biochem J. 2006 Nov 15;400(1):63-73.

13.

The normal phenotype of Pmm1-deficient mice suggests that Pmm1 is not essential for normal mouse development.

Cromphout K, Vleugels W, Heykants L, Schollen E, Keldermans L, Sciot R, D'Hooge R, De Deyn PP, von Figura K, Hartmann D, Körner C, Matthijs G.

Mol Cell Biol. 2006 Aug;26(15):5621-35.

14.

Targeted disruption of the mouse phosphomannomutase 2 gene causes early embryonic lethality.

Thiel C, Lübke T, Matthijs G, von Figura K, Körner C.

Mol Cell Biol. 2006 Aug;26(15):5615-20.

15.

LAMP-2 deficient mice show depressed cardiac contractile function without significant changes in calcium handling.

Stypmann J, Janssen PM, Prestle J, Engelen MA, Kögler H, Lüllmann-Rauch R, Eckardt L, von Figura K, Landgrebe J, Mleczko A, Saftig P.

Basic Res Cardiol. 2006 Jul;101(4):281-91. Epub 2006 Apr 8.

PMID:
16604439
16.

A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme.

Roeser D, Preusser-Kunze A, Schmidt B, Gasow K, Wittmann JG, Dierks T, von Figura K, Rudolph MG.

Proc Natl Acad Sci U S A. 2006 Jan 3;103(1):81-6. Epub 2005 Dec 20.

17.

The early vertebrate Danio rerio Mr 46000 mannose-6-phosphate receptor: biochemical and functional characterisation.

Koduru S, Vegiraju SR, Nadimpalli SK, von Figura K, Pohlmann R, Dennes A.

Dev Genes Evol. 2006 Mar;216(3):133-43. Epub 2005 Dec 16.

PMID:
16362416
18.

Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease).

Koike M, Shibata M, Waguri S, Yoshimura K, Tanida I, Kominami E, Gotow T, Peters C, von Figura K, Mizushima N, Saftig P, Uchiyama Y.

Am J Pathol. 2005 Dec;167(6):1713-28.

19.

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation.

Willenborg M, Schmidt CK, Braun P, Landgrebe J, von Figura K, Saftig P, Eskelinen EL.

J Lipid Res. 2005 Dec;46(12):2559-69. Epub 2005 Sep 21.

20.

Identification of novel lysosomal matrix proteins by proteome analysis.

Kollmann K, Mutenda KE, Balleininger M, Eckermann E, von Figura K, Schmidt B, Lübke T.

Proteomics. 2005 Oct;5(15):3966-78.

PMID:
16145712
21.

Neurocognitive and psychotiform behavioral alterations and enhanced hippocampal long-term potentiation in transgenic mice displaying neuropathological features of human alpha-mannosidosis.

D'Hooge R, Lüllmann-Rauch R, Beckers T, Balschun D, Schwake M, Reiss K, von Figura K, Saftig P.

J Neurosci. 2005 Jul 13;25(28):6539-49.

22.

Proteolytic degradation of glutamate decarboxylase mediates disinhibition of hippocampal CA3 pyramidal cells in cathepsin D-deficient mice.

Shimizu T, Hayashi Y, Yamasaki R, Yamada J, Zhang J, Ukai K, Koike M, Mine K, von Figura K, Peters C, Saftig P, Fukuda T, Uchiyama Y, Nakanishi H.

J Neurochem. 2005 Aug;94(3):680-90. Epub 2005 Jun 30.

23.

Autophagic vacuoles with sarcolemmal features delineate Danon disease and related myopathies.

Sugie K, Noguchi S, Kozuka Y, Arikawa-Hirasawa E, Tanaka M, Yan C, Saftig P, von Figura K, Hirano M, Ueno S, Nonaka I, Nishino I.

J Neuropathol Exp Neurol. 2005 Jun;64(6):513-22.

PMID:
15977643
24.

Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme.

Dierks T, Dickmanns A, Preusser-Kunze A, Schmidt B, Mariappan M, von Figura K, Ficner R, Rudolph MG.

Cell. 2005 May 20;121(4):541-552. doi: 10.1016/j.cell.2005.03.001.

25.

Expression, localization, structural, and functional characterization of pFGE, the paralog of the Calpha-formylglycine-generating enzyme.

Mariappan M, Preusser-Kunze A, Balleininger M, Eiselt N, Schmidt B, Gande SL, Wenzel D, Dierks T, von Figura K.

J Biol Chem. 2005 Apr 15;280(15):15173-9. Epub 2005 Feb 11.

26.

Crystal structure of human pFGE, the paralog of the Calpha-formylglycine-generating enzyme.

Dickmanns A, Schmidt B, Rudolph MG, Mariappan M, Dierks T, von Figura K, Ficner R.

J Biol Chem. 2005 Apr 15;280(15):15180-7. Epub 2005 Feb 1.

27.

Molecular characterization of the human Calpha-formylglycine-generating enzyme.

Preusser-Kunze A, Mariappan M, Schmidt B, Gande SL, Mutenda K, Wenzel D, von Figura K, Dierks T.

J Biol Chem. 2005 Apr 15;280(15):14900-10. Epub 2005 Jan 18.

28.

Unimpaired allorejection of cells deficient for the mannose 6-phosphate receptors Mpr300 and Mpr46.

Dressel R, von Figura K, Günther E.

Transplantation. 2004 Sep 15;78(5):758-61.

PMID:
15371683
29.

Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study.

Roces DP, Lüllmann-Rauch R, Peng J, Balducci C, Andersson C, Tollersrud O, Fogh J, Orlacchio A, Beccari T, Saftig P, von Figura K.

Hum Mol Genet. 2004 Sep 15;13(18):1979-88. Epub 2004 Jul 21.

PMID:
15269179
30.

Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts.

Eskelinen EL, Schmidt CK, Neu S, Willenborg M, Fuertes G, Salvador N, Tanaka Y, Lüllmann-Rauch R, Hartmann D, Heeren J, von Figura K, Knecht E, Saftig P.

Mol Biol Cell. 2004 Jul;15(7):3132-45. Epub 2004 Apr 30.

31.

Granzyme-mediated cytotoxicity does not involve the mannose 6-phosphate receptors on target cells.

Dressel R, Raja SM, Höning S, Seidler T, Froelich CJ, von Figura K, Günther E.

J Biol Chem. 2004 May 7;279(19):20200-10. Epub 2004 Feb 25.

32.

Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik.

Schwarz M, Thiel C, Lübbehusen J, Dorland B, de Koning T, von Figura K, Lehle L, Körner C.

Am J Hum Genet. 2004 Mar;74(3):472-81. Epub 2004 Feb 16.

33.

Deficiency of mannose 6-phosphate receptors and lysosomal storage: a morphometric analysis of hepatocytes of neonatal mice.

Schellens JP, Saftig P, von Figura K, Everts V.

Cell Biol Int. 2003;27(11):897-902.

PMID:
14585283
34.
35.

Thyroid functions of mouse cathepsins B, K, and L.

Friedrichs B, Tepel C, Reinheckel T, Deussing J, von Figura K, Herzog V, Peters C, Saftig P, Brix K.

J Clin Invest. 2003 Jun;111(11):1733-45.

36.

Multiple sulfatase deficiency is caused by mutations in the gene encoding the human C(alpha)-formylglycine generating enzyme.

Dierks T, Schmidt B, Borissenko LV, Peng J, Preusser A, Mariappan M, von Figura K.

Cell. 2003 May 16;113(4):435-44.

37.

A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis.

Thiel C, Schwarz M, Peng J, Grzmil M, Hasilik M, Braulke T, Kohlschütter A, von Figura K, Lehle L, Körner C.

J Biol Chem. 2003 Jun 20;278(25):22498-505. Epub 2003 Apr 8.

38.

Identification of the putative mannose 6-phosphate receptor (MPR 46) protein in the invertebrate mollusc.

Nadimpalli SK, von Figura K.

Biosci Rep. 2002 Oct-Dec;22(5-6):513-21.

PMID:
12635848
39.

LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice.

Gamp AC, Tanaka Y, Lüllmann-Rauch R, Wittke D, D'Hooge R, De Deyn PP, Moser T, Maier H, Hartmann D, Reiss K, Illert AL, von Figura K, Saftig P.

Hum Mol Genet. 2003 Mar 15;12(6):631-46.

PMID:
12620969
40.

Identification of formylglycine in sulfatases by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry.

Peng J, Schmidt B, von Figura K, Dierks T.

J Mass Spectrom. 2003 Jan;38(1):80-6.

PMID:
12526009
41.

Locating the anomalous scatterer substructures in halide and sulfur phasing.

Usón I, Schmidt B, von Bülow R, Grimme S, von Figura K, Dauter M, Rajashankar KR, Dauter Z, Sheldrick GM.

Acta Crystallogr D Biol Crystallogr. 2003 Jan;59(Pt 1):57-66. Epub 2002 Dec 19.

PMID:
12499540
42.

Posttranslational modification of serine to formylglycine in bacterial sulfatases. Recognition of the modification motif by the iron-sulfur protein AtsB.

Marquordt C, Fang Q, Will E, Peng J, von Figura K, Dierks T.

J Biol Chem. 2003 Jan 24;278(4):2212-8. Epub 2002 Nov 4.

43.

Structural requirements for interactions between leucine-sorting signals and clathrin-associated adaptor protein complex AP3.

Rodionov DG, Höning S, Silye A, Kongsvik TL, von Figura K, Bakke O.

J Biol Chem. 2002 Dec 6;277(49):47436-43. Epub 2002 Oct 4.

44.

The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts.

Hartmann D, de Strooper B, Serneels L, Craessaerts K, Herreman A, Annaert W, Umans L, Lübke T, Lena Illert A, von Figura K, Saftig P.

Hum Mol Genet. 2002 Oct 1;11(21):2615-24.

PMID:
12354787
45.

Role of LAMP-2 in lysosome biogenesis and autophagy.

Eskelinen EL, Illert AL, Tanaka Y, Schwarzmann G, Blanz J, Von Figura K, Saftig P.

Mol Biol Cell. 2002 Sep;13(9):3355-68.

46.

Deficiency of dolichyl-P-Man:Man7GlcNAc2-PP-dolichyl mannosyltransferase causes congenital disorder of glycosylation type Ig.

Thiel C, Schwarz M, Hasilik M, Grieben U, Hanefeld F, Lehle L, von Figura K, Körner C.

Biochem J. 2002 Oct 1;367(Pt 1):195-201.

47.

The polarized epithelia-specific mu 1B-adaptin complements mu 1A-deficiency in fibroblasts.

Eskelinen EL, Meyer C, Ohno H, von Figura K, Schu P.

EMBO Rep. 2002 May;3(5):471-7. Epub 2002 Apr 18.

48.

Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId.

Hansske B, Thiel C, Lübke T, Hasilik M, Höning S, Peters V, Heidemann PH, Hoffmann GF, Berger EG, von Figura K, Körner C.

J Clin Invest. 2002 Mar;109(6):725-33.

49.

Phosphorylation of the AP2 mu subunit by AAK1 mediates high affinity binding to membrane protein sorting signals.

Ricotta D, Conner SD, Schmid SL, von Figura K, Honing S.

J Cell Biol. 2002 Mar 4;156(5):791-5. Epub 2002 Mar 4.

50.

The tyrosine motifs of Lamp 1 and LAP determine their direct and indirect targetting to lysosomes.

Obermüller S, Kiecke C, von Figura K, Höning S.

J Cell Sci. 2002 Jan 1;115(Pt 1):185-94.

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