NGLY1-Deficiency and the Non-Lysosomal Degradation of Free <i>N</i>-Glycans

• Suzuki Tadashi

  Glycometabolic Biochemistry Laboratory, RIKEN Cluster for Pioneering Research

抄録

Peptide:N-glycanase (PNGase) is a deglycosylating enzyme that acts on asparagine-linked (N-linked) glycans. I was involved in the discovery of this enzyme activity in 1993, as well as the identification of the gene encoding this enzyme in 2000. In 2007, I proposed the existence of a novel “non-lysosomal” glycan degradation pathway in which cytoplasmic PNGase plays pivotal roles. In 2008, I was fortunate to receive a Young Investigator Award from the Japanese Society of Carbohydrate Research. Since that time remarkable progress has been made regarding the functional analysis of the cytoplasmic PNGase (NGLY1 in mammals). After the discovery of a human genetic disorder, an NGLY1-deficiency, caused by the genetic mutations of NGLY1 gene, the functional importance of this enzyme has attracted widespread interest. In this article, I briefly summarize recent research progress on NGLY1.

収録刊行物

• Ｔｒｅｎｄｓ　ｉｎ　Ｇｌｙｃｏｓｃｉｅｎｃｅ　ａｎｄ　Ｇｌｙｃｏｔｅｃｈｎｏｌｏｇｙ

  Ｔｒｅｎｄｓ　ｉｎ　Ｇｌｙｃｏｓｃｉｅｎｃｅ　ａｎｄ　Ｇｌｙｃｏｔｅｃｈｎｏｌｏｇｙ 31 (181), SE55-SE56, 2019-07-25

  FCCA(Forum: Carbohydrates Coming of Age)

キーワード

• peptide:<i>N</i>-glycanase
• non-lysosomal glycan degradation
• <i>N</i>-glycans
• ENGase
• NGLY1-deficiency

詳細情報

• CRID: 1390845713084477312
• NII論文ID: 130007683657
• DOI: 10.4052/tigg.1926.2se
• ISSN: 18832113
• ISSN: 09157352
• Web Site: https://www.jstage.jst.go.jp/article/tigg/31/181/31_1926.2SE/_pdf
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• 抄録ライセンスフラグ: 使用不可