Gene Symbol |
NGLY1
|
Entrez Gene |
55768
|
Alt Symbol |
CDG1V, PNG1, PNGase
|
Species |
Human
|
Gene Type |
protein-coding
|
Description |
N-glycanase 1
|
Other Description |
hPNGase|peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase|peptide:N-glycanase
|
Swissprots |
Q6PJD8 Q96IV0 Q9BVR8 Q59FB1 Q9NR70 B4DJE9
|
Accessions |
EAW64363 EAW64364 EAW64365 EAW64366 Q96IV0 AB114414 AB114415 AB209549 BAD92786 AF250924 AAF74720 AK001867 BAA91950 AK022471 AK296047 BAG58811 AK310473 AL832748 BC000963 AAH00963 BC007226 AAH07226 BC017220 AAH17220 BQ017148 DC329733 DQ893509 ABM84435 DQ896501 ABM87500 XM_005265316 XP_005265373 XM_005265317 XP_005265374 XM_011533944 XP_011532246 XM_011533945 XP_011532247 XR_940470 XR_940471 NM_001145293 NP_001138765 NM_001145294 NP_001138766 NM_001145295 NP_001138767 NM_018297 NP_060767 NM_025105
|
Function |
Specifically deglycosylates the denatured form of N- linked glycoproteins in the cytoplasm and assists their proteasome-mediated degradation. Cleaves the beta-aspartyl- glucosamine (GlcNAc) of the glycan and the amide side chain of Asn, converting Asn to Asp. Prefers proteins containing high- mannose over those bearing complex type oligosaccharides. Can recognize misfolded proteins in the endoplasmic reticulum that are exported to the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is a prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins. {ECO:0000269|PubMed:14749736, ECO:0000269|PubMed:15358861}.
|
Subcellular Location |
Cytoplasm {ECO:0000269|PubMed:15358861}.
|
Top Pathways |
Protein processing in endoplasmic reticulum
|