Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction
The cytosolic enzyme NGLY1 (N-glycanase 1) is a central mediator of glycoprotein catabolism. The enzyme acts to cleave N-linked glycans from modified substrate asparagine residues prior to degradation of misfolded proteins by the proteasome, playing a key and well-conserved role in the ER-associated...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2023-12-01
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| Series: | Autophagy Reports |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/27694127.2023.2166324 |
| _version_ | 1797685699151921152 |
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| author | Holger B. R. Kramer Sarah Ann Allman |
| author_facet | Holger B. R. Kramer Sarah Ann Allman |
| author_sort | Holger B. R. Kramer |
| collection | DOAJ |
| description | The cytosolic enzyme NGLY1 (N-glycanase 1) is a central mediator of glycoprotein catabolism. The enzyme acts to cleave N-linked glycans from modified substrate asparagine residues prior to degradation of misfolded proteins by the proteasome, playing a key and well-conserved role in the ER-associated degradation/ERAD pathway. In a clinical context, NGLY1 disorder represents a rare congenital disorder of deglycosylation where mutations in the NGLY1 gene result in the loss of enzyme function. Patients with NGLY1 disorder present with a broad and varied array of symptoms, which can include moderate to profound levels of developmental delay, seizures, and complex movement disorders, as well as alacrima. Our recent results highlight a causal link between NGLY1 inhibition and macroautophagy/autophagy induction. |
| first_indexed | 2024-03-12T00:55:04Z |
| format | Article |
| id | doaj.art-47c5ee5fc2fd4679a6bcdc0d52a8a50f |
| institution | Directory Open Access Journal |
| issn | 2769-4127 |
| language | English |
| last_indexed | 2024-03-12T00:55:04Z |
| publishDate | 2023-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Autophagy Reports |
| spelling | doaj.art-47c5ee5fc2fd4679a6bcdc0d52a8a50f2023-09-14T13:24:40ZengTaylor & Francis GroupAutophagy Reports2769-41272023-12-012110.1080/27694127.2023.21663242166324Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy inductionHolger B. R. Kramer0Sarah Ann Allman1University of OxfordReading School of Pharmacy, University of ReadingThe cytosolic enzyme NGLY1 (N-glycanase 1) is a central mediator of glycoprotein catabolism. The enzyme acts to cleave N-linked glycans from modified substrate asparagine residues prior to degradation of misfolded proteins by the proteasome, playing a key and well-conserved role in the ER-associated degradation/ERAD pathway. In a clinical context, NGLY1 disorder represents a rare congenital disorder of deglycosylation where mutations in the NGLY1 gene result in the loss of enzyme function. Patients with NGLY1 disorder present with a broad and varied array of symptoms, which can include moderate to profound levels of developmental delay, seizures, and complex movement disorders, as well as alacrima. Our recent results highlight a causal link between NGLY1 inhibition and macroautophagy/autophagy induction.http://dx.doi.org/10.1080/27694127.2023.2166324autophagyautophagy inductioncaspase inhibitioneradglycoprotein catabolismngly1peptide:n-glycanasez-vad-fmk |
| spellingShingle | Holger B. R. Kramer Sarah Ann Allman Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction Autophagy Reports autophagy autophagy induction caspase inhibition erad glycoprotein catabolism ngly1 peptide:n-glycanase z-vad-fmk |
| title | Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction |
| title_full | Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction |
| title_fullStr | Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction |
| title_full_unstemmed | Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction |
| title_short | Sugar coating autophagy: exploring the links between the inhibition of NGLY1 (N-glycanase 1) and autophagy induction |
| title_sort | sugar coating autophagy exploring the links between the inhibition of ngly1 n glycanase 1 and autophagy induction |
| topic | autophagy autophagy induction caspase inhibition erad glycoprotein catabolism ngly1 peptide:n-glycanase z-vad-fmk |
| url | http://dx.doi.org/10.1080/27694127.2023.2166324 |
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