Article
Loss of peptide:N-glycanase causes proteasome dysfunction mediated by a sugar-recognizing ubiquitin ligase.
Proceedings of the National Academy of Sciences of the United States of America - 6 Jul 2021
Yoshida Yukiko, Asahina Makoto, Murakami Arisa, Kawawaki Junko, Yoshida Meari, Fujinawa Reiko, Iwai Kazuhiro, Tozawa Ryuichi, Matsuda Noriyuki, Tanaka Keiji, Suzuki Tadashi
Abstract excerpt
Mutations in the human peptide:N-glycanase gene (NGLY1), which encodes a cytosolic de-N-glycosylating enzyme, cause a congenital autosomal recessive disorder. In rodents, the loss of Ngly1 results in severe developmental delay or lethality, but the underlying mechanism remains unknown. In this study, we found that deletion of Fbxo6 (also known as Fbs2), which encodes a ubiquitin ligase subunit that recognizes...
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