Identification |
HMDB Protein ID
| CDBP04652 |
Secondary Accession Numbers
| Not Available |
Name
| Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase |
Description
| Not Available |
Synonyms
|
- N-glycanase 1
- PNGase
- Peptide:N-glycanase
- 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
|
- Cytoplasm
|
Pathways
|
Not Available
|
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
|
|
Transmembrane Regions
|
|
Protein Sequence
|
>Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
MAAAALGSSSGSASPAVAELCQNTPETFLEASKLLLTYADNILRNPNDEKYRSIRIGNTA
FSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAIERSSRLDGSNKSH
KVKSSQQPAASTQLPTTPSSNPSGLNQHTRNRQGQSSDPPSASTVAADSAILEVLQSNIQ
HVLVYENPALQEKALACIPVQELKRKSQEKLSRARKLDKGINISDEDFLLLELLHWFKEE
FFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEK
LLETRCGRCGEWANCFTLCCRAVGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCD
KPLLYEIGWGKKLSYVIAFSKDEVVDVTWRYSCKHEEVIARRTKVKEALLRDTINGLNKQ
RQLFLSENRRKELLQRIIVELVEFISPKTPKPGELGGRISGSVAWRVARGEMGLQRKETL
FIPCENEKISKQLHLCYNIVKDRYVRVSNNNQTISGWENGVWKMESIFRKVETDWHMVYL
ARKEGSSFAYISWKFECGSVGLKVDSISIRTSSQTFQTGTVEWKLRSDTAQVELTGDNSL
HSYADFSGATEVILEAELSRGDGDVAWQHTQLFRQSLNDHEENCLEIIIKFSDL
|
External Links |
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 |