LIMP-2 Expression Is Critical for Β-Glucocerebrosidase Activity and Α-Synuclein Clearance
LIMP-2 expression is critical for β-glucocerebrosidase activity and α-synuclein clearance Michelle Rothauga,1, Friederike Zunkea,1, Joseph R. Mazzullib, Michaela Schweizerc, Hermann Altmeppend, Renate Lüllmann-Rauche, Wouter W. Kallemeijnf, Paulo Gasparg, Johannes M. Aertsf, Markus Glatzeld, Paul Saftiga, Dimitri Kraincb, Michael Schwakea,h, and Judith Blanza,2 aInstitute of Biochemistry and eAnatomical Institute, Christian Albrechts University of Kiel, 24098 Kiel, Germany; bDepartment of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611; cDepartment of Electron Microscopy, Centre for Molecular Neurobiology, and dInstitute of Neuropathology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany; fDepartment of Medical Biochemistry, Academic Medical Centre, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands; gUnidade de Biologia do Lisossoma e do Peroxissoma, Instituto de Biologia Molecular e Celular, 4150-180 Porto, Portugal; and hFaculty of Chemistry/Biochemistry III, University of Bielefeld, 33615 Bielefeld, Germany Edited by Thomas C. Südhof, Stanford University School of Medicine, Stanford, CA, and approved September 15, 2014 (received for review April 4, 2014) Mutations within the lysosomal enzyme β-glucocerebrosidase (GC) transgenic mouse models of GD and PD (16). Moreover, recent data result in Gaucher disease and represent a major risk factor for suggestthataccumulatedα-syn disrupts ER/Golgi trafficking of GC, developing Parkinson disease (PD). Loss of GC activity leads to
[Show full text]