Impacts of N-glycanase1 (NGLY1) Down Regulation on the Function of Mitochondria.

Authors

Yanwen Chen, Ruijie Liu, Shaoxing Zhang, Yuxin Zhang, Qiange Lin, Yilin Ye, Shuying Yuan, Xinrong Lu, Linfei Wang, Li Chen, Guiqin Sun

Published in

Journal of cellular biochemistry. Volume 127. Issue 8. Pages e70119.

Abstract

N-glycanase 1 (NGLY1) is involved in intracellular misfolded protein degradation, releasing a de-N-glycosylated protein and a complete N-oligosaccharide. Enzymatic defects in NGLY1 may cause NGLY1-related congenital disorder of deglycosylation (NGLY1-CDDG). NGLY1 patients exhibit cognition and coordination defects, and the regulatory impact of NGLY1 in the organism deserves in-depth investigation. In this study, we established NGLY1-knockdown human foreskin fibroblasts-1 (HFF-1) cells and observed mitochondrial function impairments. We conservatively suggest that NGLY1 has global regulatory roles within cells. The Calnexin/IP3R/VDAC1 axis acts as a communication bridge and represents one mechanism underlying NGLY1-mediated modulation of mitochondrial function. This has significant implications for addressing the clinical disease problems presented by NGLY1-CDDG.

PMID:
42634536
Bibliographic data and abstract were imported from PubMed on 24 Aug 2026.

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