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Gene Review

GNS  -  glucosamine (N-acetyl)-6-sulfatase

Homo sapiens

Synonyms: G6S, Glucosamine-6-sulfatase, N-acetylglucosamine-6-sulfatase
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Disease relevance of GNS

  • Deficiency of G6S results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome) [1].
  • Mucopolysaccharidosis type IIID (MPS IIID; Sanfilippo syndrome type D; MIM 252940) is caused by deficiency of the activity of N-acetylglucosamine-6-sulfatase (GNS), which is normally required for degradation of heparan sulfate [2].
  • These results indicate that the direct inoculation method of Vitek GNS cards from Enterobacteriaceae bloodstream infections (detected by Bactec 9240, Becton-Dickinson, Cockeysville, MD, USA) performed as well as the NCCLS broth microdilution test [3].
  • Clinical evaluation of the Vitek automated system with cards GNS 122 and 127 and VTK-R07.01 software for antimicrobial susceptibility testing of Pseudomonas aeruginosa [4].

High impact information on GNS

  • The IDS sequence has strong sequence homology with other sulfatases (such as sea urchin arylsulfatase, human arylsulfatases A, B, and C, and human glucosamine 6-sulfatase), suggesting that the sulfatases comprise an evolutionarily related family of genes that arose by gene duplication and divergent evolution [5].
  • Both forms of caprine MPS IIID result from a nonsense mutation and consequent deficiency of lysosomal N-acetylglucosamine 6-sulfatase (G6S) activity and are associated with tissue storage and urinary excretion of heparan sulfate (HS) [6].
  • For instance, the amino acid motifs SNG, ENN, LNG, and LNN were found to be more prone to deamidation, whereas the motifs GNT, TNY, YNP, WNS, SNF, CNV, SNT, WNS, FNW, HNA, FNS, SNK, GNV, HNH, SNY, LNW, SNL, NNF, DNA, GNS, and FNR showed no deamidation [7].
  • The binding of chicken and mammalian viruses to tracheal epithelial cells of green monkey decreased after treatment of cells with glucosamine-6-sulfatase suggesting the presence of 6-O-Su-3'SLN determinants in the airway epithelium [8].
  • Genomic basis of mucopolysaccharidosis type IIID (MIM 252940) revealed by sequencing of GNS encoding N-acetylglucosamine-6-sulfatase [2].

Biological context of GNS


Anatomical context of GNS

  • Furthermore, deficient activity of G6S was confirmed in cultured skin fibroblasts [11].

Associations of GNS with chemical compounds

  • Glucosamine-6-sulphatase (G6S), a lysosomal enzyme found in all cells, is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate [1].
  • MATERIALS AND METHODS: In a 2-year period 253 sildenafil nonresponders were evaluated by the same urologist (GNS) [12].

Other interactions of GNS


Analytical, diagnostic and therapeutic context of GNS

  • Regional mapping by in situ hybridization of a 3H-labelled human G6S cDNA probe to human metaphase chromosomes indicated that the G6S gene is localized to chromosome 12 at q14 [1].
  • METHODS: We measured serum IGF-I and IGFBPs levels by radioimmune assay and immune radiomagnetic assay in 36 children with NS, consisting of an active stage group (ANS, n = 12), a remission stage group (RE, n = 12), an active stage group with glucocorticoid treatment (GNS, n = 12), and a normal control group (NC, n = 10) [14].


  1. Chromosomal localization of the gene for human glucosamine-6-sulphatase to 12q14. Robertson, D.A., Callen, D.F., Baker, E.G., Morris, C.P., Hopwood, J.J. Hum. Genet. (1988) [Pubmed]
  2. Genomic basis of mucopolysaccharidosis type IIID (MIM 252940) revealed by sequencing of GNS encoding N-acetylglucosamine-6-sulfatase. Mok, A., Cao, H., Hegele, R.A. Genomics (2003) [Pubmed]
  3. Accuracy of the Vitek system for antimicrobial susceptibility testing Enterobacteriaceae bloodstream infection isolates: use of "direct" inoculation from Bactec 9240 blood culture bottles. Putnam, L.R., Howard, W.J., Pfaller, M.A., Koontz, F.P., Jones, R.N. Diagn. Microbiol. Infect. Dis. (1997) [Pubmed]
  4. Clinical evaluation of the Vitek automated system with cards GNS 122 and 127 and VTK-R07.01 software for antimicrobial susceptibility testing of Pseudomonas aeruginosa. Chandler, L.J., Poulter, M., Reisner, B., Woods, G. Diagn. Microbiol. Infect. Dis. (2002) [Pubmed]
  5. Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA. Wilson, P.J., Morris, C.P., Anson, D.S., Occhiodoro, T., Bielicki, J., Clements, P.R., Hopwood, J.J. Proc. Natl. Acad. Sci. U.S.A. (1990) [Pubmed]
  6. Caprine mucopolysaccharidosis-IIID: clinical, biochemical, morphological and immunohistochemical characteristics. Jones, M.Z., Alroy, J., Boyer, P.J., Cavanagh, K.T., Johnson, K., Gage, D., Vorro, J., Render, J.A., Common, R.S., Leedle, R.A., Lowrie, C., Sharp, P., Liour, S.S., Levene, B., Hoard, H., Lucas, R., Hopwood, J.J. J. Neuropathol. Exp. Neurol. (1998) [Pubmed]
  7. Identification and characterization of deamidation sites in the conserved regions of human immunoglobulin gamma antibodies. Chelius, D., Rehder, D.S., Bondarenko, P.V. Anal. Chem. (2005) [Pubmed]
  8. H5N1 chicken influenza viruses display a high binding affinity for Neu5Acalpha2-3Galbeta1-4(6-HSO3)GlcNAc-containing receptors. Gambaryan, A.S., Tuzikov, A.B., Pazynina, G.V., Webster, R.G., Matrosovich, M.N., Bovin, N.V. Virology (2004) [Pubmed]
  9. Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase. Tomatsu, S., Fukuda, S., Masue, M., Sukegawa, K., Fukao, T., Yamagishi, A., Hori, T., Iwata, H., Ogawa, T., Nakashima, Y. Biochem. Biophys. Res. Commun. (1991) [Pubmed]
  10. Complex genome organization in the GNS-L intergenic region of Adelaide River rhabdovirus. Wang, Y., McWilliam, S.M., Cowley, J.A., Walker, P.J. Virology (1994) [Pubmed]
  11. The ultrastructure of skin from a patient with mucopolysaccharidosis IIID. Alroy, J., Jones, M.Z., Rutledge, J.C., Taylor, J.W., Toone, J., Applegarth, D., Hopwood, J.J. Acta Neuropathol. (1997) [Pubmed]
  12. Salvage of sildenafil failures referred from primary care physicians. Atiemo, H.O., Szostak, M.J., Sklar, G.N. J. Urol. (2003) [Pubmed]
  13. Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase. Robertson, D.A., Freeman, C., Nelson, P.V., Morris, C.P., Hopwood, J.J. Biochem. Biophys. Res. Commun. (1988) [Pubmed]
  14. Effect of glucocorticoid treatment on insulin like growth factor-I and its binding proteins in children with nephrotic syndrome. Dong, F., Zhou, X., Pang, N., Wei, M. Chin. Med. J. (2002) [Pubmed]
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