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Please use this identifier to cite or link to this item: http://hdl.handle.net/1942/10987

Title: The ceramide transporter and the Goodpasture antigen binding protein: one protein - one function?
Authors: Mencarelli, Chiara
Losen, Mario
Hammels, Caroline
De Vry, Jochen
Hesselink, Matthijs K. C.
Steinbusch, Harry W. M.
Martinez-Martinez, Pilar
Issue Date: 2010
Citation: JOURNAL OF NEUROCHEMISTRY, 113 (6). p. 1369-1386
Abstract: P>The Goodpasture antigen-binding protein (GPBP) and its splice variant the ceramide transporter (CERT) are multifunctional proteins that have been found to play important roles in brain development and biology. However, the function of GPBP and CERT is controversial because of their involvement in two apparently unrelated research fields: GPBP was initially isolated as a protein associated with collagen IV in patients with the autoimmune disease Goodpasture syndrome. Subsequently, a splice variant lacking a serine-rich domain of 26 amino acids (GPBP Delta 26) was found to mediate the cytosolic transport of ceramide and was therefore (re)named CERT. The two splice forms likely carry out different functions in specific sub-cellular localizations. Selective GPBP knockdown induces extensive apoptosis and tissue loss in the brain of zebrafish. GPBP/GPBP Delta 26 knock-out mice die as a result of structural and functional defects in endoplasmic reticulum and mitochondria. Because both mitochondria and ceramide play an important role in many biological events that regulate neuronal differentiation, cellular senescence, proliferation and cell death, we propose that GPBP and CERT are pivotal in neurodegenerative processes. In this review, we discuss the current state of knowledge on GPBP and CERT, including the molecular and biochemical characterization of GPBP in the field of autoimmunity as well as the fundamental research on CERT in ceramide transport, biosynthesis, localization, metabolism and cell homeostasis.
Notes: [Mencarelli, Chiara; Losen, Mario; Hammels, Caroline; De Vry, Jochen; Steinbusch, Harry W. M.; De Baets, Marc H.; Martinez-Martinez, Pilar] Maastricht Univ, Sch Mental Hlth & Neurosci, Dept Neurosci, Fac Hlth Med & Life Sci, NL-6200 MD Maastricht, Netherlands. [Hesselink, Matthijs K. C.] Maastricht Univ, NUTRIM Sch Nutr Toxicol & Metab, Dept Human Movement Sci, NL-6200 MD Maastricht, Netherlands. [De Baets, Marc H.] Hasselt Univ, Biomed Res Inst BIOMED, Neuroimmunol Grp, Diepenbeek, Belgium. p.martinez@maastrichtuniversity.nl
URI: http://hdl.handle.net/1942/10987
DOI: 10.1111/j.1471-4159.2010.06673.x
ISI #: 000278309900002
ISSN: 0022-3042
Category: A1
Type: Journal Contribution
Validation: ecoom, 2011
Appears in Collections: Research publications

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