Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Peptide: N-Glycanase 1 and Its Relationship with Congenital Disorder of Deglycosylation

Version 1 : Received: 9 March 2022 / Approved: 10 March 2022 / Online: 10 March 2022 (12:51:22 CET)

A peer-reviewed article of this Preprint also exists.

Miao, X.; Wu, J.; Chen, H.; Lu, G. Comprehensive Analysis of the Structure and Function of Peptide:N-Glycanase 1 and Relationship with Congenital Disorder of Deglycosylation. Nutrients 2022, 14, 1690. Miao, X.; Wu, J.; Chen, H.; Lu, G. Comprehensive Analysis of the Structure and Function of Peptide:N-Glycanase 1 and Relationship with Congenital Disorder of Deglycosylation. Nutrients 2022, 14, 1690.

Journal reference: Nutrients 2022, 14, 1690
DOI: 10.3390/nu14091690

Abstract

The cytosolic PNGase (peptide:N-glycanase; Png1 in yeast; NGLY1/Ngly1 in human/mice), also known as peptide-N4-(N-acetyl-beta-glucosaminyl)-asparagine ami-dase, is a well-conserved deglycosylation enzyme (EC 3.5.1.52) which catalyzes the non-lysosomal hydrolysis of an N(4)-(acetyl-β-D-glucosaminyl) asparagine residue into N-acetyl-β-D-glucosaminylamine and a peptide containing an aspartate residue. This enzyme (NGLY1) plays essential roles in clearance of misfolded or unassembled gly-coproteins through a process named ER-associated degradation (ERAD). Accumulating evidence also points out that NGLY1 deficiency can cause an autosomal recessive hu-man genetic disorder associated with abnormal development and congenital disorder of deglycosylation. In addition, the loss of NGLY1 can affect multiple cellular pathways, including but not limited to NFE2L1 pathway, Creb1/Atf1-AQP pathway, BMP path-way, AMPK pathway, and SLC12A2 ion transporter, which might be the underlying reasons for a constellation of clinical phenotypes of NGLY1 deficiency. The current comprehensive review indeed uncovers the detailed NGLY1’s structure and its im-portant roles for participation in ERAD, involvement in CDDG and potential treatment for NGLY1 deficiency.

Keywords

N-glycosylation; NGLY1; ER associated degradation process; congenital disorder of deglycosylation; NFE2L1

Subject

LIFE SCIENCES, Genetics

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