N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase

Details

Name
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase
Synonyms
  • 3.1.4.45
  • Mannose 6-phosphate-uncovering enzyme
  • Phosphodiester alpha-GlcNAcase
Gene Name
NAGPA
Organism
Humans
Amino acid sequence
>lcl|BSEQ0037182|N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase
MATSTGRWLLLRLALFGFLWEASGGLDSGASRDDDLLLPYPRARARLPRDCTRVRAGNRE
HESWPPPPATPGAGGLAVRTFVSHFRDRAVAGHLTRAVEPLRTFSVLEPGGPGGCAARRR
ATVEETARAADCRVAQNGGFFRMNSGECLGNVVSDERRVSSSGGLQNAQFGIRRDGTLVT
GYLSEEEVLDTENPFVQLLSGVVWLIRNGSIYINESQATECDETQETGSFSKFVNVISAR
TAIGHDRKGQLVLFHADGQTEQRGINLWEMAEFLLKQDVVNAINLDGGGSATFVLNGTLA
SYPSDHCQDNMWRCPRQVSTVVCVHEPRCQPPDCHGHGTCVDGHCQCTGHFWRGPGCDEL
DCGPSNCSQHGLCTETGCRCDAGWTGSNCSEECPLGWHGPGCQRPCKCEHHCPCDPKTGN
CSVSRVKQCLQPPEATLRAGELSFFTRTAWLALTLALAFLLLISTAANLSLLLSRAERNR
RLHGDYAYHPLQEMNGEPLAAEKEQPGGAHNPFKD
Number of residues
515
Molecular Weight
56072.49
Theoretical pI
6.7
GO Classification
Functions
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase activity
Processes
carbohydrate metabolic process / cellular protein modification process / lysosome organization / protein glycosylation / protein targeting to lysosome
Components
Golgi cisterna membrane / integral component of membrane
General Function
N-acetylglucosamine-1-phosphodiester alpha-n-acetylglucosaminidase activity
Specific Function
Catalyzes the second step in the formation of the mannose 6-phosphate targeting signal on lysosomal enzyme oligosaccharides by removing GlcNAc residues from GlcNAc-alpha-P-mannose moieties, which are formed in the first step. Also hydrolyzes UDP-GlcNAc, a sugar donor for Golgi N-acetylglucosaminyltransferases.
Pfam Domain Function
Transmembrane Regions
449-469
Cellular Location
Golgi apparatus
Gene sequence
>lcl|BSEQ0011632|N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase (NAGPA)
ATGGCGACCTCCACGGGTCGCTGGCTTCTCCTCCGGCTTGCACTATTCGGCTTCCTCTGG
GAAGCGTCCGGCGGCCTCGACTCGGGGGCCTCCCGCGACGACGACTTGCTACTGCCCTAT
CCACGCGCGCGCGCGCGCCTCCCCCGGGACTGCACACGGGTGCGCGCCGGCAACCGCGAG
CACGAGAGTTGGCCTCCGCCTCCCGCGACTCCCGGCGCCGGCGGTCTGGCCGTGCGCACC
TTCGTGTCGCACTTCAGGGACCGCGCGGTGGCCGGCCACCTGACGCGGGCCGTTGAGCCC
CTGCGCACCTTCTCGGTGCTGGAGCCCGGTGGACCCGGCGGCTGCGCGGCGAGACGACGC
GCCACCGTGGAGGAGACGGCGCGGGCGGCCGACTGCCGTGTCGCCCAGAACGGCGGCTTC
TTCCGCATGAACTCGGGCGAGTGCCTGGGGAACGTGGTGAGCGACGAGCGGCGGGTGAGC
AGCTCCGGGGGGCTGCAGAACGCGCAGTTCGGGATCCGCCGCGACGGGACCCTGGTCACC
GGGTACCTGTCTGAGGAGGAGGTGCTGGACACTGAGAACCCATTTGTGCAGCTGCTGAGT
GGGGTCGTGTGGCTGATTCGTAATGGAAGCATCTACATCAACGAGAGCCAAGCCACAGAG
TGTGACGAGACACAGGAGACAGGTTCCTTTAGCAAATTTGTGAATGTGATATCAGCCAGG
ACGGCCATTGGCCACGACCGGAAAGGGCAGCTGGTGCTCTTTCATGCAGACGGCCAAACG
GAGCAGCGTGGCATCAACCTGTGGGAAATGGCGGAGTTCCTGCTGAAACAGGACGTGGTC
AACGCCATCAACCTGGATGGGGGTGGCTCTGCCACCTTTGTGCTCAACGGGACCTTGGCC
AGTTACCCGTCAGATCACTGCCAGGACAACATGTGGCGCTGTCCCCGCCAAGTGTCCACC
GTGGTGTGTGTGCACGAACCCCGCTGCCAGCCGCCTGACTGCCACGGCCACGGGACCTGC
GTGGACGGGCACTGCCAATGCACCGGGCACTTCTGGCGGGGTCCCGGCTGTGATGAGCTG
GACTGTGGCCCCTCTAACTGCAGCCAGCACGGACTGTGCACGGAGACCGGCTGCCGCTGT
GATGCCGGATGGACCGGGTCCAACTGCAGTGAAGAGTGTCCCCTTGGCTGGCATGGGCCG
GGCTGCCAGAGGCCTTGTAAGTGTGAGCACCATTGTCCCTGTGACCCCAAGACTGGCAAC
TGCAGCGTCTCCAGAGTAAAGCAGTGTCTCCAGCCACCTGAAGCCACCCTGAGGGCGGGA
GAACTCTCCTTTTTCACCAGGACCGCCTGGCTAGCCCTCACCCTGGCGCTGGCCTTCCTC
CTGCTGATCAGCACTGCAGCAAACCTGTCCTTGCTCCTGTCCAGAGCAGAGAGGAACCGG
CGCCTGCATGGGGACTATGCATACCACCCGCTGCAGGAGATGAACGGGGAGCCTCTGGCC
GCAGAGAAGGAGCAGCCAGGGGGCGCCCACAACCCCTTCAAGGACTGA
Chromosome Location
16
Locus
16p13.3
External Identifiers
ResourceLink
UniProtKB IDQ9UK23
UniProtKB Entry NameNAGPA_HUMAN
GenBank Protein ID6425040
GenBank Gene IDAF187072
GenAtlas IDNAGPA
HGNC IDHGNC:17378
General References
  1. Kornfeld R, Bao M, Brewer K, Noll C, Canfield W: Molecular cloning and functional expression of two splice forms of human N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase. J Biol Chem. 1999 Nov 12;274(46):32778-85. [Article]
  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]
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  4. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  5. Do H, Lee WS, Ghosh P, Hollowell T, Canfield W, Kornfeld S: Human mannose 6-phosphate-uncovering enzyme is synthesized as a proenzyme that is activated by the endoprotease furin. J Biol Chem. 2002 Aug 16;277(33):29737-44. Epub 2002 Jun 10. [Article]
  6. Kang C, Riazuddin S, Mundorff J, Krasnewich D, Friedman P, Mullikin JC, Drayna D: Mutations in the lysosomal enzyme-targeting pathway and persistent stuttering. N Engl J Med. 2010 Feb 25;362(8):677-85. doi: 10.1056/NEJMoa0902630. Epub 2010 Feb 10. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00141N-Acetylglucosamineapproved, investigational, nutraceuticalunknownDetails