Research Topics
Genomes and Genes | Jürgen RothSummaryAffiliation: University Hospital Country: Switzerland Publications
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Publications
Tubules of the trans Golgi apparatus visualized by immunoelectron microscopyJ Roth
Department of Pathology, University of Zurich, Switzerland
Histochem Cell Biol 109:545-53. 1998..These buds and vesicle are therefore involved in constitutive exocytosis...
Protein N-glycosylation along the secretory pathway: relationship to organelle topography and function, protein quality control, and cell interactionsJürgen Roth
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Chem Rev 102:285-303. 2002
The importance of trimming reactions on asparagine-linked oligosaccharides for protein quality controlJürgen Roth
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland
Histochem Cell Biol 117:159-69. 2002....
Sodium 4-phenylbutyrate acts as a chemical chaperone on misfolded myocilin to rescue cells from endoplasmic reticulum stress and apoptosisGary Hin Fai Yam
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Schmelzbergstrasse 12, CH 8091 Zurich, Switzerland
Invest Ophthalmol Vis Sci 48:1683-90. 2007....
Protein quality control: the who's who, the where's and therapeutic escapesJürgen Roth
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, 8091 Zurich, Switzerland
Histochem Cell Biol 129:163-77. 2008....
Expression of mutant Ins2C96Y results in enhanced tubule formation causing enlargement of pre-Golgi intermediates of CHO cellsJing Yu Fan
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091, Zurich, Switzerland
Histochem Cell Biol 128:161-73. 2007..We conclude that misfolded proinsulin causes enlargement of pre-Golgi intermediates which indicates their involvement in protein quality control...
4-Phenylbutyrate rescues trafficking incompetent mutant alpha-galactosidase A without restoring its functionalityGary Hin Fai Yam
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Biochem Biophys Res Commun 360:375-80. 2007..Because of lack of functionality of rescued mutant alpha-galactosidase A, 4-phenylbutyrate seems to be of limited use as a chemical chaperone for Fabry disease...
Endomannosidase undergoes phosphorylation in the Golgi apparatusTania Torossi
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Glycobiology 20:55-61. 2010..Treatment of cells with brefeldin A blocked the posttranslational modification of endomannosidase, suggesting that phosphorylation is occurring in the Golgi apparatus, the residence of endomannosidase...
Pharmacological chaperone corrects lysosomal storage in Fabry disease caused by trafficking-incompetent variantsGary Hin Fai Yam
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Am J Physiol Cell Physiol 290:C1076-82. 2006..Pharmacological chaperones acting on misfolded, unstable mutant proteins that exhibit residual biological activity offer a convenient and cost-efficient therapeutic strategy...
The role of glucosidase II and endomannosidase in glucose trimming of asparagine-linked oligosaccharidesJürgen Roth
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Switzerland
Biochimie 85:287-94. 2003..The mutually exclusive subcellular distribution of glucosidase II and endomannosidase are discussed in terms of their significance for quality control of protein folding and N-glycosylation...
EDEM1 reveals a quality control vesicular transport pathway out of the endoplasmic reticulum not involving the COPII exit sitesChristian Zuber
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Proc Natl Acad Sci U S A 104:4407-12. 2007....
A cell culture system for the induction of Mallory bodies: Mallory bodies and aggresomes represent different types of inclusion bodiesKiyoko Hirano
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Schmelzbergstrasse 12, 8091, Zurich, Switzerland
Histochem Cell Biol 132:293-304. 2009..Thus, cultured clone 9 hepatocytes are a useful system to study further aspects of the pathobiology of MBs...
Misfolded proinsulin accumulates in expanded pre-Golgi intermediates and endoplasmic reticulum subdomains in pancreatic beta cells of Akita miceChristian Zuber
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Zurich, Switzerland
FASEB J 18:917-9. 2004....
Aggregated myocilin induces russell bodies and causes apoptosis: implications for the pathogenesis of myocilin-caused primary open-angle glaucomaGary Hin Fai Yam
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091 Zurich, Switzerland
Am J Pathol 170:100-9. 2007....
A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorderGary Hin Fai Yam
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Zurich, Switzerland
FASEB J 19:12-8. 2005..Small molecule chemical chaperones will be therapeutically useful for various lysosomal storage disorders as well as for other genetic metabolic disorders caused by mutant but nonetheless catalytically active enzymes...
Ceruloplasmin carries the anionic glycan oligo/poly alpha2,8 deaminoneuraminic acidMartin Ziak
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, Switzerland
Biochem Biophys Res Commun 295:597-602. 2002..Thus, ceruloplasmin, in addition to megalin, represents a glycoprotein carrying oligo/poly alpha2,8 KDN...
Combined comparative genomic hybridization and genomic microarray for detection of gene amplifications in pulmonary artery intimal sarcomas and adrenocortical tumorsJianming Zhao
Department of Pathology, University of Zurich, Zurich, Switzerland
Genes Chromosomes Cancer 34:48-57. 2002....
Absence of somatic SDHD mutations in sporadic neuroendocrine tumors and detection of two germline variants in paraganglioma patientsAurel Perren
Department of Pathology, University Hospital, 8091 Zurich, Switzerland
Oncogene 21:7605-8. 2002..However, LOH alone could lead to a complete loss of function since SDHD is an imprinted gene. Furthermore, we describe two germline variants possibly causing hereditary paragangliomas...
The proteasome is involved in the degradation of different aquaporin-2 mutants causing nephrogenic diabetes insipidusKiyoko Hirano
Department of Pathology, Division of Cell and Molecular Pathology, University of Zurich, Zurich, Switzerland
Am J Pathol 163:111-20. 2003..This implies the involvement of different protein quality control processes in the processing of these AQP2 mutants...
Recent progress in histochemistryChristian Zuber
Division of Cell and Molecular Pathology, Department of Pathology, University of Zurich, CH 8091, Zurich, Switzerland
Histochem Cell Biol 128:557-94. 2007..This review will highlight some advancements made in these fields...
