Ontology highlight
ABSTRACT:
SUBMITTER: Chan CB
PROVIDER: S-EPMC2626726 | biostudies-literature | 2009 Jan
REPOSITORIES: biostudies-literature
Chan Chi-Bun CB Abe Michiyo M Hashimoto Noriyoshi N Hao Chunhai C Williams Ifor R IR Liu Xia X Nakao Shinji S Yamamoto Akitsugu A Zheng Chengyun C Henter Jan-Inge JI Meeths Marie M Nordenskjold Magnus M Li Shi-Yong SY Hara-Nishimura Ikuko I Asano Masahide M Ye Keqiang K
Proceedings of the National Academy of Sciences of the United States of America 20081223 2
Asparaginyl endopeptidase (AEP or legumain) is a lysosomal cysteine protease that cleaves protein substrates on the C-terminal side of asparagine. AEP plays a pivotal role in the endosome/lysosomal degradation system and is implicated in antigen processing. The processing of the lysosomal proteases cathepsins in kidney is completely defective in AEP-deficient mice with accumulation of macromolecules in the lysosomes, which is typically seen in lysosomal disorders. Here we show that mutant mice l ...[more]