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Proteolytic processing of the neuronal ceroid lipofuscinosis related lysosomal protein CLN5.

Exp Cell Res.. 2015-10;  338(1):45-53
Silva BD, Adams J, Lee SY. Biochemistry and Molecular Biophysics Graduate Group, Kansas State University, Manhattan, KS 66506, USA.
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摘要

CLN5 is a soluble lysosomal glycoprotein. Deficiency in CLN5 protein causes neuronal ceroid lipofuscinosis, an inherited neurodegenerative lysosomal storage disorder. The function of CLN5 and how it affects lysosome activity are unclear. We identified two forms of the CLN5 protein present in most of the cell lines studied. The molecular mass difference between these two forms is about 4kDa. The fibroblast cells derived from two CLN5 patients lack both forms. Using transient transfection, we showed one of these two forms is a proprotein and the other is a C-terminal cleaved mature form. Using cycloheximide chase analysis, we were able to demonstrate that the C-terminal processing occurs post-translationally. By ... More

关键词

CLN5; Lysosomal storage disorder; Neuronal ceroid lipofuscinosis; Proteolytic processing