CREG: a novel lysosomal protein
Abstract
In eukaryotic cells, dedicated subcellular structures referred to as lysosomes play a pivotal role in the metabolism of cellular macromolecules. These organelles contain a large array of hydrolases and auxiliary proteins required for efficient substrate breakdown. Deficiencies in individual lysosomal proteins result in the gradual accumulation of undigested macromolecules which ultimately interferes with the normal functions of these compartments and the affected cells. In humans, about 45 lysosomal storage diseases have been directly linked to the deficiency of one or several lysosomal proteins. However, the molecular etiology of some lysosomal storage diseases is still unknown. Since lysosomal dysfunction has been also observed in other human disease states including neurodegenerative diseases and cancer, considerable efforts have been recently undertaken to establish the complete proteome of lysosomes. This has led to the discovery of a range of novel lysosomal proteins with as yet unknown functions. This project aims at a thorough characterization of a particularly striking novel lysosomal protein, Cellular Repressor of E1A-stimulated Genes (CREG). The cellular functions of CREG in mammals are a matter of debate. The occurrence of CREG in non-mammalian species such as plants and insects is not understood. Hence, the main goals of this study are to assess the consequences of CREG ablation in mammalian cells and whole organisms, to screen the lysosomal proteome for possible interaction partners of the protein, to elucidate the relationship between CREG's unique structural features and its biological activity, and to characterize the structural and functional properties of CREG from plants and insects as compared to their mammalian counterparts. The detailed characterization of CREG and its cellular tasks will improve our understanding of the biology of lysosomes and related compartments. It also has the potential to provide novel insights into the pathophysiology of human diseases associated with disturbed functions of these organelles.
lysosome protein transport glycoprotein receptor storage disease
Publikationen
Cellular repressor of E1A-stimulated genes is a bona fide lysosomal protein which undergoes proteolytic maturation during its biosynthesis.
Autoren: Schähs, P; Weidinger, P; Probst, OC; Svoboda, B; Stadlmann, J; Beug, H; Waerner, T; Mach, L Jahr: 2008
Journal articles
Cellular repressor of E1A-stimulated genes is a bona fide lysosomal protein which undergoes proteolytic maturation during its biosynthesis
Autoren: Schähs, P., Weidinger, P., Probst, O.C., Svoboda, B., Stadlmann, J., Beug, H., Waerner, T., Mach, L. Jahr: 2008
Conference & Workshop proceedings, paper, abstract
Project staff
Lukas Mach
Univ.Prof. Dipl.-Ing. Dr. Lukas Mach
lukas.mach@boku.ac.at
Tel: +43 1 47654-94065, 94360
Project Leader
01.06.2008 - 31.05.2013