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Physiological importance of NGLY1, as revealed by rodent model analyses.
Fujihira, Haruhiko; Asahina, Makoto; Suzuki, Tadashi.
Afiliação
  • Fujihira H; Glycometabolic Biochemistry Laboratory, RIKEN Cluster for Pioneering Research, RIKEN, Saitama, 3510198, Japan.
  • Asahina M; Division of Glycobiologics, Intractable Disease Research Center, Juntendo University Graduate School of Medicine, Tokyo, 1138421, Japan.
  • Suzuki T; T-CiRA Discovery, Takeda Pharmaceutical Company Ltd, Kanagawa, 2518555, Japan.
J Biochem ; 171(2): 161-167, 2022 Feb 21.
Article em En | MEDLINE | ID: mdl-34580715
Cytosolic peptide:N-glycanase (NGLY1) is an enzyme that cleaves N-glycans from glycoproteins that has been retrotranslocated from the endoplasmic reticulum (ER) lumen into the cytosol. It is known that NGLY1 is involved in the degradation of cytosolic glycans (non-lysosomal glycan degradation) as well as ER-associated degradation, a quality control system for newly synthesized glycoproteins. The discovery of NGLY1 deficiency, which is caused by mutations in the human NGLY1 gene and results in multisystemic symptoms, has attracted interest in the physiological functions of NGLY1 in mammals. Studies using various animal models led to the identification of possible factors that contribute to the pathogenesis of NGLY1 deficiency. In this review, we summarize phenotypic consequences that have been reported for various Ngly1-deficient rodent models and discuss future perspectives to provide more insights into the physiological functions of NGLY1.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Roedores / Degradação Associada com o Retículo Endoplasmático Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Revista: J Biochem Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Japão

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Roedores / Degradação Associada com o Retículo Endoplasmático Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Revista: J Biochem Ano de publicação: 2022 Tipo de documento: Article País de afiliação: Japão