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dr. P.Q.Q. (Judith) Smissen

dr. P.Q.Q. (Judith) Smissen

Full Professor
dr. P.Q.Q. (Judith) Smissen
  • Cell Biology

Biography

Biography

Since 2001 Judith Klumperman is professor of Cell Biology and since 2008 chair of the Department of Cell Biology at the UMC Utrecht. She is also head of the Cell Microscopy Core of the UMC Utrecht, which is widely recognized as one of the best expertise centers in the world for immuno-electron microscopy. On a yearly basis the UMC Utrechts trains and assist over 40 scientists from the Netherlands and 15-20 international guests.She is often asked for expert microscopy advise and participate(d) in a large number of international collaborations.

Her research focus on understanding the molecular basis of human diseases, with an emphasis on diseases of the endo-lysosomal system. Diseases specifically addressed in my work are: Lysosomal storage disorders (Pompe disease; Mucolipidosis II/I-Cell disease) – Alzheimer disease – Hermansky Pudlak Syndrome (pigmentation bleeding disorder) - Cancer – virus and bacterial infections – ARC syndrome –Microvillar inclusion Disease. In addition, her work has elucidated fundamental concepts of endosome and lysosome biogenesis, of which the most recent finding is a novel pathway for the delivery of lysosomal membrane proteins to the lysosomes.

Side Activities

Regular (>10) member or chair of Appointment Advisory Committee’s (Benoemings Advies Commissies)  for new professors (Utrecht, Nijmegen, Wageningen and Groningen University

Regular (>20) member of chair of thesis assessment committee’s (Universities of Utrecht, Leiden, Rotterdam, Amsterdam, Nijmegen, Leuven (Belgium), Copenhagen (Denmark)).

ESFRI initiative EuroBioImaging. 2010-2014: Steering Committee (member). 2009-2014: WP4: Coordinator for Correlative Light Electron Microscopy.

Utrecht Graduate School Institute of Biomembranes, (interim project leader, Board Member, Chair)  2000-2010

Netherlands Society for Microscopy (NVvM) Board member, Vice president 2001-2007 

Member project management team NL-BioImaging-AM, the Dutch Research Infrastructure for Advanced Microscopy.   2001-2007

Royal Dutch Academy of Arts and Sciences (KNAW).  Financing Biomedical Research  and Council for Medical Sciences and  Royal Dutch Academy of Arts and Sciences (KNAW). Chair exploratory committee ‘Tissue Engineering’  2002-2011

committee NWO medium-sized investment grants and Big Investments  2001-2015

University Utrecht delegation to improve contact with Chinese universities 2006

Regional committee Asia, University of Utrecht  2006-2010

ZON-MW (Medical Research Netherlands – Medical Sciences) -committee medium-sized (<9k€) investment grants. Member and Vice-chair 2005-2009

Utrecht PhD program ‘Biomembranes’. 2004-2006: Board member. 2007-2010: Chair. 2010 – present: Board member

Member committee ‘Internationalisering II’. UMC Utrecht   2008-2011

Chair committee Electron microscopy University Utrecht  2011

Coordinator CLEM workpackage ESFRI infrastructure EuroBioimaging  2009-2015

Member user committees STW (foundation for technical sciences) in projects of Meijering (Erasmus MC), Gerritsen (UU), Giepmans (UMC Groningen) and Koster (Leiden UMC)  2012-2018

Member international review panel Cell Biology and Biophysics Unit European Molecular Biology Laboratory (EMBL), Heidelberg, Germany 2013

Chair ad hoc committee scientific integrity, installed by B. vd Zwaan, rector University Utrecht  2013-2014

vice-National Contact person (NCP) for the Netherlands in EuroBioImaging   2009-2015

Member project management team NL-BioImaging-AM, the Dutch Research Infrastructure for Advanced Microscopy.  2001-1015

NL-BioImaging-AM. Dutch Roadmap initiative for Research Infrastructure for Advanced Microscopy. 2011-present: Vice-chair of the Project Management Team

Dutch Technology Centre (DTL).2011-present: Member of the Core group

Associate editor: Molecular Biology of the Cell. Editorial boards: Traffic, Histochemistry and Cell Biology, Biology of the Cell, PeerJ. Section editor: Current Opinion in Cell Biology. Member of FACULTY F1000

Cell Microscopy Centre (CMC), UMC Utrecht,  Chair (2009-2018)

Steering Committee EM Square, Utrecht University   (2015 - present)

Netherlands Electron Microscopy Infrastructure (NEMI) Chair Executive Board (2015 - present)

Center for Molecular Medicine (CMM) UMC Utrecht, member management team (2018 - present) 

Editorships

Traffic, Histochemistry and Cell Biology, Biology of the Cell, Current Opinion in Cell Biology (Section editor of the Membranes and organelles issue in 2004 and 2021), Molecular Biology of the Cell (associate editor till 2018), PeerJ (Open access journal of The Royal Society)

August 2021:  Guest editor Current Opinion in Cell Biology

Reviewer: Cell, Nature, Journal of Cell Biology, EMBO Journal, Nature Cell Biology, Nature Communications, Cell Reports, Molecular Biology of the Cell, Journal of Cell Science, Traffic, Histochemistry & Cell Biology.

Grant reviewer: Dutch Society for Scientific research (NWO), Wellcome Trust (UK), Medical Research Council (UK), Telethon (Italy), KNAW, Koningin Wilhelmina Fonds (KWF), EMBO

Fellowship and Awards

2007: 20th Foundation lecture, Center for Cell and Molecular Biology, Hyderabad, India.

2004: VICI Award (1.25M€) from the Netherlands Organisation for Scientific Research.

1998: UMC Utrecht award: Attraction and maintenance of female talent.

Research Output (161)

Author Correction: Vps3 and Vps8 control integrin trafficking from early to recycling endosomes and regulate integrin-dependent functions

Jonker Caspar T H, Galmes Romain, Veenendaal Tineke, Ten Brink Corlinda, van der Welle Reini E N, Liv Nalan, de Rooij Johan, Peden Andrew A, van der Sluijs Peter, Margadant Coert, Klumperman Judith Dec 2021, In: Nature Communications. 12 , p. 1-1

ER - lysosome contacts at a pre-axonal region regulate axonal lysosome availability

Özkan Nazmiye, Koppers Max, van Soest Inge, van Harten Alexandra, Jurriens Daphne, Liv Nalan, Klumperman Judith, Kapitein Lukas C, Hoogenraad Casper C, Farías Ginny G Dec 2021, In: Nature Communications. 12 , p. 1-18

Fatty acylation enhances the cellular internalization and cytosolic distribution of a cystine-knot peptide

Gao Xinxin, Mazière Ann De, Beard Rhiannon, Klumperman Judith, Hannoush Rami N 19 Nov 2021, In: iScience. 24 , p. 1-17

Erratum: DGAT2 partially compensates for lipid-induced ER stress in human DGAT1-deficient intestinal stem cells

van Rijn Jorik M, van Hoesel Marliek, de Heus Cecilia, van Vugt Anke H M, Klumperman Judith, Nieuwenhuis Edward E S, Houwen Roderick H J, Middendorp Sabine 6 Oct 2021, In: Journal of Lipid Research. 62 , p. 1-1

The stress-sensing domain of activated IRE1 alpha forms helical filaments in narrow ER membrane tubes

Tran Ngoc-Han, Carter Stephen D, De Mazière Ann, Ashkenazi Avi, Klumperman Judith, Walter Peter, Jensen Grant J 1 Oct 2021, In: Science. 374 , p. 52-57 6 p.

Understanding membrane traffic from molecular ensemble, energetics, and the cell biology of participant components

Klumperman Judith, Pucadyil Thomas Aug 2021, In: Current Opinion in Cell Biology. 71 , p. iii-vi

A-Synuclein fibrils subvert lysosome structure and function for the propagation of protein misfolding between cells through tunneling nanotubes

Dilsizoglu Senol Aysegul, Samarani Maura, Syan Sylvie, Guardia Carlos M, Nonaka Takashi, Liv Nalan, Latour-Lambert Patricia, Hasegawa Masato, Klumperman Judith, Bonifacino Juan S, Zurzolo Chiara Jul 2021, In: PLoS Biology. 19 , p. 1-49

Cysteamine-bicalutamide combination therapy corrects proximal tubule phenotype in cystinosis

Jamalpoor Amer, van Gelder Charlotte Agh, Yousef Yengej Fjodor A, Zaal Esther A, Berlingerio Sante P, Veys Koenraad R, Pou Casellas Carla, Voskuil Koen, Essa Khaled, Ammerlaan Carola Me, Rega Laura Rita, van der Welle Reini En, Lilien Marc R, Rookmaaker Maarten B, Clevers Hans, Klumperman Judith, Levtchenko Elena, Berkers Celia R, Verhaar Marianne C, Altelaar Maarten, Masereeuw Rosalinde, Janssen Manoe J 24 Jun 2021, In: Embo Molecular Medicine. 13 , p. 1-20

Neurodegenerative VPS41 variants inhibit HOPS function and mTORC1-dependent TFEB/TFE3 regulation

van der Welle Reini E N, Jobling Rebekah, Burns Christian, Sanza Paolo, van der Beek Jan A, Fasano Alfonso, Chen Lan, Zwartkruis Fried J, Zwakenberg Susan, Griffin Edward F, Ten Brink Corlinda, Veenendaal Tineke, Liv Nalan, van Ravenswaaij-Arts Conny M A, Lemmink Henny H, Pfundt Rolph, Blaser Susan, Sepulveda Carolina, Lozano Andres M, Yoon Grace, Santiago-Sim Teresa, Asensio Cedric S, Caldwell Guy A, Caldwell Kim A, Chitayat David, Klumperman Judith 14 Apr 2021, In: Embo Molecular Medicine. 13 24 p.

Pancreatic beta-Cell-Specific Deletion of VPS41 Causes Diabetes Due to Defects in Insulin Secretion

Burns Christian H, Yau Belinda, Rodriguez Anjelica, Triplett Jenna, Maslar Drew, An You Sun, van der Welle Reini E N, Kossina Ross G, Fisher Max R, Strout Gregory W, Bayguinov Peter O, Veenendaal Tineke, Chitayat David, Fitzpatrick James A J, Klumperman Judith, Kebede Melkam A, Asensio Cedric S 1 Feb 2021, In: Diabetes. 70 , p. 436-448 13 p.

All research output

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