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NGLY1

Gene
NGLY1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Homo sapiens
Length
654 amino acids
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.
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
74.39 kDa
Sequence
MAAAALGSSSGSASPAVAELCQNTPETFLEASKLLLTYADNILRNPNDEKYRSIRIGNTAFSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAIERSSRLDGSNKSHKVKSSQQPAASTQLPTTPSSNPSGLNQHTRNRQGQSSDPPSASTVAADSAILEVLQSNIQHVLVYENPALQEKALACIPVQELKRKSQEKLSRARKLDKGINISDEDFLLLELLHWFKEEFFHWVNNVLCSKCGGQTRSRDRSLLPSDDELKWGAKEVEDHYCDACQFSNRFPRYNNPEKLLETRCGRCGEWANCFTLCCRAVGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCDKPLLYEIGWGKKLSYVIAFSKDEVVDVTWRYSCKHEEVIARRTKVKEALLRDTINGLNKQRQLFLSENRRKELLQRIIVELVEFISPKTPKPGELGGRISGSVAWRVARGEMGLQRKETLFIPCENEKISKQLHLCYNIVKDRYVRVSNNNQTISGWENGVWKMESIFRKVETDWHMVYLARKEGSSFAYISWKFECGSVGLKVDSISIRTSSQTFQTGTVEWKLRSDTAQVELTGDNSLHSYADFSGATEVILEAELSRGDGDVAWQHTQLFRQSLNDHEENCLEIIIKFSDL

Gene
NGLY1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Macaca fascicularis
Length
654 amino acids
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 (By similarity).
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
74.524 kDa
Sequence
MAAAALGSSSGSASPAVAELCQNTPETFLEASKLLLTYADNILRNPNDEKYRSIRIGNTAFSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAIERSSRLDGSNKSHKVESSQQPAASTQLPTTPSSNPSGLNQHTRNRQGQSPDPPSASTVTPDSAILGVLQSNIQHVLVYENPALQEKALACIPVQELKRKSQEKLSRVRKLDKGTNISDEDFLLLELLHWFKEEFFHWVNNILCSKCGGQTRSRDRSLLPNDDELKWGAKKVEDHYCDACQFSNRFPRYNNPEKLLETRCGRCGEWANCFTLCCRALGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCDKPLLYEIGWGKKLSYVIAFSKDEVVDVTWRYSCKHEEVIARRTKVKEALLRETINGLNKQRQLFLSENRRKELLQRIIVELVEFISPKTPKPGELGGRISGSVAWRVARGEMGLQRKETSFIPSENEKISKQLHLCYNIVKDRYVRVSNNNQTISGWENGVWKMESIFRKVETDWHMVYLARKEGSSFAYISWKFECGSVGLKIDSISIRTTSQTFQTGTIEWKLRSDTARVELTGDNNLHSYADFSGATEVILEAELSRGDGDVAWQHTQLFRQSLNDHEENCLEIIIKFSDL

Gene
NGLY1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Gallus gallus
Length
651 amino acids
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 (By similarity).
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
73.903 kDa
Sequence
MAGSAGLSSSPSSPAVRELCKNARDTFLEASRLLLTYADNILRNPYEEKYRSIRNGNPAFSTRLLPVRGAVECLFEMGFQEGETHMVFPKEASIEQLRKVRDLIAVERSSRLNESNQVHRSASSETVAITQAIAHQPSRPAGSVPTPDHQQPEPSLLQSLKMAADILTTLQSKCDHLILAYESTSLQQKALALIPLQQLKEKAQRKLAQATRLDKGEHVNEEDFLLLELLNWFKNDFFHWVDNLPCSRCGGQTEGKRDYLSPTDDDLRWNADRVENHYCSQCQFCNRFPRYNNPEKLLETRRGRCGEWANCFTLCCRAVGFEARYVRDWTDHVWTEVYSASQKRWLHCDPCENVCDKPLLYEIGWGKKLSYVIAFSKDEVVDVTWRYSCKHQEVLTRRTALSEAKLRETINAINKKKQQSLSEGRRKELLERTIVELAEFISPKTPKPGEFGGRTSGSMAWRIARGETGSEERKEVIFIPSEKEKASKLFHLMYNVVEDSYTRISNNNEQITGWGTGIWKAESIARKVETDWKMVYLARKEGSSSASISWKFECKSVGLKIDNISIRTSSQTFHSGRIKWRLYSPTAEVILVGDNSLSSYSDFCGATEVTLEATLSGGDGKAAWQHTQLFRNSLAGCGENCLEMIIKLADL

Gene
Ngly1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Mus musculus
Length
651 amino acids
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.
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
74.275 kDa
Sequence
MASATLGSSSSSASPAVAELCQNTPETFLEASKLLLTYADNILRNPSDEKYRSIRIGNTAFSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAIERSSRLDGSSKKVQFSQHPAAAKLPLEQSEDPAGLIRHSGNQTGQLPSLPSAPMVVGDSTILKVLQSNIQHVQLYENPVLQEKALTCIPVSELKRKAQEKLFRARKLDKGTNVSDEDFLLLELLHWFKEEFFRWVNNIVCSKCGGETRSRDEALLPNDDELKWGAKNVENHYCDACQLSNRFPRYNNPEKLLETRCGRCGEWANCFTLCCRALGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCDKPLLYEIGWGKKLSYIIAFSKDEVVDVTWRYSCKHDEVMSRRTKVKEELLRETINGLNKQRQLSLSESRRKELLQRIIVELVEFISPKTPRPGELGGRVSGSLAWRVARGETGLERKEILFIPSENEKISKQFHLRYDIVRDRYIRVSDNNINISGWENGVWKMESIFRKVEKDWNMVYLARKEGSSFAYISWKFECGSAGLKVDTVSIRTSSQSFESGSVRWKLRSETAQVNLLGDKNLRSYNDFSGATEVTLEAELSRGDGDVAWQHTQLFRQSLNDSGENGLEIIITFNDL

Gene
Ngly1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Rattus norvegicus
Length
651 amino acids
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 (By similarity).
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
74.677 kDa
Sequence
MASATLGSSSSSASPAVAELCQNTPETFLEASKLLLTYADNILRNPSDEKYRSIRIGNTAFSTRLLPVRGAVECLFEMGFEEGETHLIFPKKASVEQLQKIRDLIAVERRSRLDGSSQKVEFSQHPAAVRLPAEQPEDPTGLMQHSGNQPGQPLSLPSAPLVVGDSTIFKVLQSNIQHVQLYENPVLQEKALACIPVNELKRKSQEKLFRARKLDKGTKVSDEDFLLLELLHWFKEEFFHWVNNVVCSRCGRETRSRDEALPPNDDELKWGAKNVEDHYCDACQLSNRFPRYNNPEKLLETRCGRCGEWANCFTLCCRALGFEARYVWDYTDHVWTEVYSPSQQRWLHCDACEDVCDKPLLYEIGWGKKLSYIIAFSKDEVVDVTWRYSCKHEEVMSRRTKVKEELLRETINGLNKQRQLLLSESRRKELLQRIIVELVEFISPKTPRPGELGGRVSGSLAWRVARGETCLERKEILFIPSENEKISKQFHLRYDIVRDRYIRVSDNNANISGWENGVWKMESIFRKVEKDWNMVYLARKEGSSFAYISWKFECGSAGLKVDNVSIRTSSQSFETGSVRWKLRSEMAQVNLLGDRNLRSYDDFCGATEVTLEAELSRGDGDVAWQHTQLFRQSLNDHAENGLEIIITFSDL

Gene
ngly1
Protein
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Organism
Danio rerio
Length
644 amino acids
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 (By similarity).
Similarity
Belongs to the transglutaminase-like superfamily. PNGase family.
Mass
72.489 kDa
Sequence
MPGSQGVLALCENTTEVFLDVSKLLITYADNILRNPNEEKYRSIRIGNPTFSTKLLPVKGAVECLFEMGFEEAETHLVFPRSASVEKLRQVRETIAAERDQRLGASNTASSPQNASVPPVPAPPPSLSSTASAGPAAAAVPTPAPASIPFTSSSATFQRTVQSNFQHVLVYEDPELQQKALECIPHELLRSRAKERLKQANDADAACSLGEEDMLVLDLLQWFKSDFFSWVDNLPCIQCGGKTQPSGSLSPSSDDLHWDAGRVENHFCHTCQLSTRFPRYNNPEKLLETRKGRCGEWANCFTLLCRALGLEARYIWDSTDHVWTEVYSQSQRRWIHCDPCENACDKPLLYEVGWGKKLSYILAFSKDQVADVTWRYSCKHPEVLSRRTQVQETWLLHMLNKLNAERQQFLGAERKQQLLQRLLVELVEFISPKSPQPGELGGRMSGSLAWRAARGETGASNAKEDTQENVFTPSESEKESKRFHICYNVTKDCYYRVSNGQETIQGWQKGAWRTENMFRKEEHDWQMVYLARTEGASFGRMSWKFNCAPAGMKMKSASVRVFSQTFHSGTVRWSLSTNETTTEFPGDGELHSFSNVSGGTELIVEAELTGGDGSTSWQHAQLFRQSLKDTEKVLFEVMVEMEKS