Liver-specific deletion of Ngly1 causes abnormal nuclear morphology and lipid metabolism under food stress.

doi:10.1016/j.bbadis.2019.165588

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Article

Liver-specific deletion of Ngly1 causes abnormal nuclear morphology and lipid metabolism under food stress.

Biochimica et biophysica acta. Molecular basis of disease - 1 Mar 2020

Fujihira Haruhiko, Masahara-Negishi Yuki, Akimoto Yoshihiro, Hirayama Hiroto, Lee Hyeon-Cheol, Story Benjamin A, Mueller William F, Jakob Petra, Clauder-Münster Sandra, Steinmetz Lars M, Radhakrishnan Senthil K, Kawakami Hayato, Kamada Yoshihiro, Miyoshi Eiji, Yokomizo Takehiko, Suzuki Tadashi

Abstract excerpt

The cytoplasmic peptide:N-glycanase (Ngly1) is a de-N-glycosylating enzyme that cleaves N-glycans from misfolded glycoproteins and is involved in endoplasmic reticulum-associated degradation. The recent discovery of NGLY1-deficiency, which causes severe systemic symptoms, drew attention to the physiological function of Ngly1 in mammals. While several studies have been carried out to reveal the physiological...

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PMID: 31733337
DOI: 10.1016/j.bbadis.2019.165588

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