Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease

Peters J, Rittger A, Weisner R, Knabbe J, Zunke F, Rothaug M, Damme M, Berkovic SF, Blanz J, Saftig P, Schwake M (2015)
Biochemical and Biophysical Research Communications 457(3): 334-340.

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Fakultät für Chemie > Biochemie III
Abstract
The lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) has been identified as a receptor for enterovirus 71 uptake and mannose-6-phosphate-independent lysosomal trafficking of the acid hydrolase beta-glucocerebrosidase. Here we show that LIMP-2 undergoes proteolytic cleavage mediated by lysosomal cysteine proteases. Heterologous expression and in vitro studies suggest that cathepsin-F is mainly responsible for the lysosomal processing of wild-type LIMP-2. Furthermore, examination of purified lysosomes revealed that LIMP-2 undergoes proteolysis in vivo. Mutations in the gene encoding cathepsin-F (CTSF) have recently been associated with type-B-Kufs-disease, an adult form of neuronal ceroid-lipofuscinosis. In this study we show that disease-causing cathepsin-F mutants fail to cleave LIMP-2. Our findings provide evidence that LIMP-2 represents an in vivo substrate of cathepsin-F with relevance for understanding the pathophysiology of type-B-Kufs-disease. (C) 2015 Elsevier Inc. All rights reserved.
Keywords
ceroid-lipofuscinosis ; Neuronal ; Lysosomal storage disease ; Cathepsin-F ; LIMP-2 ; Kufs disease
Publishing Year
2015
ISSN
0006-291X
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Peters J, Rittger A, Weisner R, et al. Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease. Biochemical and Biophysical Research Communications. 2015;457(3):334-340.
Peters, J., Rittger, A., Weisner, R., Knabbe, J., Zunke, F., Rothaug, M., Damme, M., et al. (2015). Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease. Biochemical and Biophysical Research Communications, 457(3), 334-340. doi:10.1016/j.bbrc.2014.12.111
Peters, J., Rittger, A., Weisner, R., Knabbe, J., Zunke, F., Rothaug, M., Damme, M., Berkovic, S. F., Blanz, J., Saftig, P., et al. (2015). Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease. Biochemical and Biophysical Research Communications 457, 334-340.
Peters, J., et al., 2015. Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease. Biochemical and Biophysical Research Communications, 457(3), p 334-340.
J. Peters, et al., “Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease”, Biochemical and Biophysical Research Communications, vol. 457, 2015, pp. 334-340.
Peters, J., Rittger, A., Weisner, R., Knabbe, J., Zunke, F., Rothaug, M., Damme, M., Berkovic, S.F., Blanz, J., Saftig, P., Schwake, M.: Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease. Biochemical and Biophysical Research Communications. 457, 334-340 (2015).
Peters, Judith, Rittger, Andrea, Weisner, Rebecca, Knabbe, Johannes, Zunke, Friederike, Rothaug, Michelle, Damme, Markus, Berkovic, Samuel F., Blanz, Judith, Saftig, Paul, and Schwake, Michael. “Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease”. Biochemical and Biophysical Research Communications 457.3 (2015): 334-340.
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