Identification
HMDB Protein ID CDBP04652
Secondary Accession Numbers Not Available
Name Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Description Not Available
Synonyms
  1. N-glycanase 1
  2. PNGase
  3. Peptide:N-glycanase
  4. hPNGase
Gene Name NGLY1
Protein Type Enzyme
Biological Properties
General Function Involved in protein binding
Specific 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 reticulun that are exported in the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins
GO Classification
Component
cell part
intracellular part
cytoplasm
Function
binding
protein binding
Process
metabolic process
catabolic process
macromolecule catabolic process
glycoprotein catabolic process
Cellular Location
  1. Cytoplasm
Pathways
Gene Properties
Chromosome Location Chromosome:3
Locus 3p24.2
SNPs NGLY1
Gene Sequence
>1965 bp
ATGGCGGCGGCGGCATTGGGCAGCTCCTCAGGCTCGGCGTCCCCGGCCGTGGCTGAGCTC
TGCCAGAACACCCCGGAGACCTTTTTGGAGGCCTCCAAGCTGCTGCTCACCTATGCTGAC
AACATCCTCAGAAACCCTAATGATGAAAAATATAGATCCATCCGGATTGGAAACACAGCC
TTTTCTACTAGACTCTTGCCTGTCAGAGGAGCTGTTGAATGTTTATTTGAAATGGGCTTT
GAAGAGGGAGAAACACATCTCATCTTTCCTAAAAAAGCTTCAGTGGAGCAGCTGCAAAAA
ATTCGTGACCTGATTGCCATAGAGAGAAGTAGCAGACTGGATGGCTCAAATAAGAGCCAC
AAAGTAAAGTCATCTCAGCAACCTGCAGCCAGTACCCAGCTTCCTACAACACCATCTTCA
AATCCCAGTGGGTTAAACCAGCACACAAGGAACCGTCAAGGGCAGTCATCAGATCCACCA
TCTGCTTCAACGGTTGCTGCTGACTCAGCCATTCTAGAAGTTCTTCAGTCCAACATTCAG
CATGTGCTGGTCTATGAAAATCCTGCTCTTCAGGAGAAAGCGTTGGCTTGTATTCCGGTC
CAAGAACTAAAAAGGAAATCACAAGAAAAGTTATCGAGAGCTAGAAAATTGGATAAAGGT
ATCAATATAAGTGATGAGGATTTTCTTTTGCTGGAGCTTTTGCACTGGTTTAAGGAAGAA
TTTTTTCACTGGGTGAATAACGTTTTGTGCAGCAAATGTGGTGGACAGACTAGGTCTAGA
GATAGATCATTACTGCCCAGTGATGATGAGCTGAAGTGGGGTGCAAAGGAAGTGGAAGAT
CATTACTGTGATGCCTGCCAGTTCAGCAATCGATTCCCAAGATATAATAACCCTGAGAAA
CTTTTGGAAACAAGATGTGGACGGTGTGGCGAGTGGGCCAATTGTTTTACACTGTGCTGC
CGAGCTGTAGGGTTTGAAGCTCGCTATGTTTGGGATTACACAGACCATGTCTGGACAGAA
GTCTATTCTCCTTCTCAGCAGCGGTGGCTGCACTGTGATGCATGTGAAGATGTCTGTGAC
AAGCCACTCCTTTATGAAATAGGATGGGGCAAGAAGCTTTCCTATGTCATAGCATTTTCA
AAAGATGAGGTAGTTGATGTCACTTGGCGATATTCCTGCAAACATGAAGAGGTGATTGCC
AGAAGAACTAAGGTTAAAGAAGCATTACTTCGAGACACTATTAATGGGCTTAATAAGCAG
AGGCAACTGTTTTTGTCAGAAAACAGAAGGAAAGAACTTCTCCAGAGGATAATTGTGGAG
CTTGTTGAATTTATATCTCCCAAAACCCCTAAACCTGGAGAACTTGGGGGAAGAATATCT
GGGTCAGTGGCTTGGAGAGTAGCCCGAGGTGAAATGGGTCTACAGAGAAAAGAAACCTTG
TTTATTCCCTGTGAAAATGAGAAGATTTCTAAACAGCTCCACCTTTGTTACAATATTGTG
AAAGATCGTTATGTTCGAGTTTCAAATAACAATCAAACCATTTCTGGATGGGAGAATGGC
GTGTGGAAAATGGAATCTATATTCAGAAAAGTTGAAACAGACTGGCACATGGTATATTTG
GCCCGAAAGGAAGGATCATCTTTTGCTTATATTTCCTGGAAGTTTGAGTGTGGGTCAGTT
GGCCTAAAAGTAGATAGCATTTCTATTAGAACAAGTAGTCAAACTTTTCAGACTGGAACA
GTAGAATGGAAATTGCGATCTGATACAGCACAAGTAGAACTGACAGGCGATAACAGTCTT
CACTCCTATGCTGATTTTTCTGGTGCCACTGAAGTTATTTTGGAAGCAGAATTAAGCAGA
GGAGATGGTGATGTCGCTTGGCAACACACCCAGCTGTTTAGACAAAGCTTAAATGACCAT
GAAGAAAATTGTTTGGAGATAATTATAAAATTCAGTGACCTTTGA
Protein Properties
Number of Residues 654
Molecular Weight 74389.4
Theoretical pI 6.88
Pfam Domain Function
Signals
  • ["None"]
Transmembrane Regions
  • ["None"]
Protein Sequence
>Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
MAAAALGSSSGSASPAVAELCQNTPETFLEASKLLLTYADNILRNPNDEKYRSIRIGNTA
FSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAIERSSRLDGSNKSH
KVKSSQQPAASTQLPTTPSSNPSGLNQHTRNRQGQSSDPPSASTVAADSAILEVLQSNIQ
HVLVYENPALQEKALACIPVQELKRKSQEKLSRARKLDKGINISDEDFLLLELLHWFKEE
FFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEK
LLETRCGRCGEWANCFTLCCRAVGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCD
KPLLYEIGWGKKLSYVIAFSKDEVVDVTWRYSCKHEEVIARRTKVKEALLRDTINGLNKQ
RQLFLSENRRKELLQRIIVELVEFISPKTPKPGELGGRISGSVAWRVARGEMGLQRKETL
FIPCENEKISKQLHLCYNIVKDRYVRVSNNNQTISGWENGVWKMESIFRKVETDWHMVYL
ARKEGSSFAYISWKFECGSVGLKVDSISIRTSSQTFQTGTVEWKLRSDTAQVELTGDNSL
HSYADFSGATEVILEAELSRGDGDVAWQHTQLFRQSLNDHEENCLEIIIKFSDL
GenBank ID Protein 21314690
UniProtKB/Swiss-Prot ID Q96IV0
UniProtKB/Swiss-Prot Entry Name NGLY1_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_018297.3
GeneCard ID NGLY1
GenAtlas ID NGLY1
HGNC ID HGNC:17646
References
General References Not Available