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Items: 1 to 50 of 69

1.

Pharmacologic manipulation of lysosomal enzyme transport across the blood-brain barrier.

Urayama A, Grubb JH, Sly WS, Banks WA.

J Cereb Blood Flow Metab. 2016 Mar;36(3):476-86. doi: 10.1177/0271678X15614589. Epub 2015 Nov 3.

2.

Alpha Adrenergic Induction of Transport of Lysosomal Enzyme across the Blood-Brain Barrier.

Urayama A, Dohgu S, Robinson SM, Sly WS, Grubb JH, Banks WA.

PLoS One. 2015 Nov 6;10(11):e0142347. doi: 10.1371/journal.pone.0142347. eCollection 2015.

3.

Correction: High Resolution Crystal Structure of Human β-Glucuronidase Reveals Structural Basis of Lysosome Targeting.

Hassan MI, Waheed A, Grubb JH, Klei HE, Korolev S, Sly WS.

PLoS One. 2015 Sep 14;10(9):e0138401. doi: 10.1371/journal.pone.0138401. eCollection 2015. No abstract available.

4.

High resolution crystal structure of human β-glucuronidase reveals structural basis of lysosome targeting.

Hassan MI, Waheed A, Grubb JH, Klei HE, Korolev S, Sly WS.

PLoS One. 2013 Nov 19;8(11):e79687. doi: 10.1371/journal.pone.0079687. eCollection 2013. Erratum in: PLoS One. 2015;10(9):e0138401.

5.

Biochemical evidence for superior correction of neuronal storage by chemically modified enzyme in murine mucopolysaccharidosis VII.

Huynh HT, Grubb JH, Vogler C, Sly WS.

Proc Natl Acad Sci U S A. 2012 Oct 16;109(42):17022-7. doi: 10.1073/pnas.1214779109. Epub 2012 Oct 1.

6.

Assessment of bone dysplasia by micro-CT and glycosaminoglycan levels in mouse models for mucopolysaccharidosis type I, IIIA, IVA, and VII.

Rowan DJ, Tomatsu S, Grubb JH, Montaño AM, Sly WS.

J Inherit Metab Dis. 2013 Mar;36(2):235-46. doi: 10.1007/s10545-012-9522-x. Epub 2012 Sep 13.

7.

Long circulating enzyme replacement therapy rescues bone pathology in mucopolysaccharidosis VII murine model.

Rowan DJ, Tomatsu S, Grubb JH, Haupt B, Montaño AM, Oikawa H, Sosa AC, Chen A, Sly WS.

Mol Genet Metab. 2012 Sep;107(1-2):161-72. doi: 10.1016/j.ymgme.2012.07.002. Epub 2012 Jul 14.

8.

Identification of N-glycans displaying mannose-6-phosphate and their site of attachment on therapeutic enzymes for lysosomal storage disorder treatment.

Bones J, Mittermayr S, McLoughlin N, Hilliard M, Wynne K, Johnson GR, Grubb JH, Sly WS, Rudd PM.

Anal Chem. 2011 Jul 1;83(13):5344-52. doi: 10.1021/ac2007784. Epub 2011 Jun 7.

PMID:
21599028
9.

New strategies for enzyme replacement therapy for lysosomal storage diseases.

Grubb JH, Vogler C, Sly WS.

Rejuvenation Res. 2010 Apr-Jun;13(2-3):229-36. doi: 10.1089/rej.2009.0920. Review.

10.

Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome).

Tomatsu S, Montaño AM, Dung VC, Grubb JH, Sly WS.

Hum Mutat. 2009 Apr;30(4):511-9. doi: 10.1002/humu.20828. Review.

11.

Infused Fc-tagged beta-glucuronidase crosses the placenta and produces clearance of storage in utero in mucopolysaccharidosis VII mice.

Grubb JH, Vogler C, Tan Y, Shah GN, MacRae AF, Sly WS.

Proc Natl Acad Sci U S A. 2008 Jun 17;105(24):8375-80. doi: 10.1073/pnas.0803715105. Epub 2008 Jun 10.

12.

Mannose 6-phosphate receptor-mediated transport of sulfamidase across the blood-brain barrier in the newborn mouse.

Urayama A, Grubb JH, Sly WS, Banks WA.

Mol Ther. 2008 Jul;16(7):1261-6. doi: 10.1038/mt.2008.84. Epub 2008 Apr 29.

13.

Acidic amino acid tag enhances response to enzyme replacement in mucopolysaccharidosis type VII mice.

Montaño AM, Oikawa H, Tomatsu S, Nishioka T, Vogler C, Gutierrez MA, Oguma T, Tan Y, Grubb JH, Dung VC, Ohashi A, Miyamoto K, Orii T, Yoneda Y, Sly WS.

Mol Genet Metab. 2008 Jun;94(2):178-89. doi: 10.1016/j.ymgme.2008.01.007. Epub 2008 Mar 21.

PMID:
18359257
14.

HFE association with transferrin receptor 2 increases cellular uptake of transferrin-bound iron.

Waheed A, Britton RS, Grubb JH, Sly WS, Fleming RE.

Arch Biochem Biophys. 2008 Jun 1;474(1):193-7. doi: 10.1016/j.abb.2008.02.041. Epub 2008 Mar 7.

PMID:
18353247
15.

Chemically modified beta-glucuronidase crosses blood-brain barrier and clears neuronal storage in murine mucopolysaccharidosis VII.

Grubb JH, Vogler C, Levy B, Galvin N, Tan Y, Sly WS.

Proc Natl Acad Sci U S A. 2008 Feb 19;105(7):2616-21. doi: 10.1073/pnas.0712147105. Epub 2008 Feb 11.

16.

Epinephrine enhances lysosomal enzyme delivery across the blood brain barrier by up-regulation of the mannose 6-phosphate receptor.

Urayama A, Grubb JH, Banks WA, Sly WS.

Proc Natl Acad Sci U S A. 2007 Jul 31;104(31):12873-8. Epub 2007 Jul 23.

17.

Characterization and pharmacokinetic study of recombinant human N-acetylgalactosamine-6-sulfate sulfatase.

Tomatsu S, Montaño AM, Gutierrez M, Grubb JH, Oikawa H, Dung VC, Ohashi A, Nishioka T, Yamada M, Yamada M, Tosaka Y, Trandafirescu GG, Orii T.

Mol Genet Metab. 2007 May;91(1):69-78. Epub 2007 Mar 2.

PMID:
17336563
18.

Enzyme therapy in mannose receptor-null mucopolysaccharidosis VII mice defines roles for the mannose 6-phosphate and mannose receptors.

Sly WS, Vogler C, Grubb JH, Levy B, Galvin N, Tan Y, Nishioka T, Tomatsu S.

Proc Natl Acad Sci U S A. 2006 Oct 10;103(41):15172-7. Epub 2006 Oct 2.

19.

Expression of membrane-bound carbonic anhydrases IV, IX, and XIV in the mouse heart.

Scheibe RJ, Gros G, Parkkila S, Waheed A, Grubb JH, Shah GN, Sly WS, Wetzel P.

J Histochem Cytochem. 2006 Dec;54(12):1379-91.

20.

Enhancement of drug delivery to bone: characterization of human tissue-nonspecific alkaline phosphatase tagged with an acidic oligopeptide.

Nishioka T, Tomatsu S, Gutierrez MA, Miyamoto K, Trandafirescu GG, Lopez PL, Grubb JH, Kanai R, Kobayashi H, Yamaguchi S, Gottesman GS, Cahill R, Noguchi A, Sly WS.

Mol Genet Metab. 2006 Jul;88(3):244-55. Epub 2006 Apr 17.

21.

Development of MPS IVA mouse (Galnstm(hC79S.mC76S)slu) tolerant to human N-acetylgalactosamine-6-sulfate sulfatase.

Tomatsu S, Gutierrez M, Nishioka T, Yamada M, Yamada M, Tosaka Y, Grubb JH, Montaño AM, Vieira MB, Trandafirescu GG, Peña OM, Yamaguchi S, Orii KO, Orii T, Noguchi A, Laybauer L.

Hum Mol Genet. 2005 Nov 15;14(22):3321-35. Epub 2005 Oct 11.

PMID:
16219627
22.

Overcoming the blood-brain barrier with high-dose enzyme replacement therapy in murine mucopolysaccharidosis VII.

Vogler C, Levy B, Grubb JH, Galvin N, Tan Y, Kakkis E, Pavloff N, Sly WS.

Proc Natl Acad Sci U S A. 2005 Oct 11;102(41):14777-82. Epub 2005 Sep 14.

23.

Carbonic anhydrase XIV identified as the membrane CA in mouse retina: strong expression in Müller cells and the RPE.

Ochrietor JD, Clamp MF, Moroz TP, Grubb JH, Shah GN, Waheed A, Sly WS, Linser PJ.

Exp Eye Res. 2005 Oct;81(4):492-500.

PMID:
16126196
24.

Defining the pathway for Tat-mediated delivery of beta-glucuronidase in cultured cells and MPS VII mice.

Orii KO, Grubb JH, Vogler C, Levy B, Tan Y, Markova K, Davidson BL, Mao Q, Orii T, Kondo N, Sly WS.

Mol Ther. 2005 Aug;12(2):345-52.

25.

Carbonic anhydrase XIV is enriched in specific membrane domains of retinal pigment epithelium, Muller cells, and astrocytes.

Nagelhus EA, Mathiisen TM, Bateman AC, Haug FM, Ottersen OP, Grubb JH, Waheed A, Sly WS.

Proc Natl Acad Sci U S A. 2005 May 31;102(22):8030-5. Epub 2005 May 18.

26.

Keratan sulphate levels in mucopolysaccharidoses and mucolipidoses.

Tomatsu S, Okamura K, Maeda H, Taketani T, Castrillon SV, Gutierrez MA, Nishioka T, Fachel AA, Orii KO, Grubb JH, Cooper A, Thornley M, Wraith E, Barrera LA, Laybauer LS, Giugliani R, Schwartz IV, Frenking GS, Beck M, Kircher SG, Paschke E, Yamaguchi S, Ullrich K, Haskins M, Isogai K, Suzuki Y, Orii T, Kondo N, Creer M, Okuyama T, Tanaka A, Noguchi A.

J Inherit Metab Dis. 2005;28(2):187-202.

PMID:
15877208
27.

Developmentally regulated mannose 6-phosphate receptor-mediated transport of a lysosomal enzyme across the blood-brain barrier.

Urayama A, Grubb JH, Sly WS, Banks WA.

Proc Natl Acad Sci U S A. 2004 Aug 24;101(34):12658-63. Epub 2004 Aug 16.

28.

Glycosylation-independent targeting enhances enzyme delivery to lysosomes and decreases storage in mucopolysaccharidosis type VII mice.

LeBowitz JH, Grubb JH, Maga JA, Schmiel DH, Vogler C, Sly WS.

Proc Natl Acad Sci U S A. 2004 Mar 2;101(9):3083-8. Epub 2004 Feb 19.

29.

Development and testing of new screening method for keratan sulfate in mucopolysaccharidosis IVA.

Tomatsu S, Okamura K, Taketani T, Orii KO, Nishioka T, Gutierrez MA, Velez-Castrillon S, Fachel AA, Grubb JH, Cooper A, Thornley M, Wraith E, Barrera LA, Giugliani R, Schwartz IV, Frenking GS, Beck M, Kircher SG, Paschke E, Yamaguchi S, Ullrich K, Isogai K, Suzuki Y, Orii T, Kondo N, Creer M, Noguchi A.

Pediatr Res. 2004 Apr;55(4):592-7. Epub 2004 Jan 7.

PMID:
14711889
30.

Expression, assay, and structure of the extracellular domain of murine carbonic anhydrase XIV: implications for selective inhibition of membrane-associated isozymes.

Whittington DA, Grubb JH, Waheed A, Shah GN, Sly WS, Christianson DW.

J Biol Chem. 2004 Feb 20;279(8):7223-8. Epub 2003 Dec 3.

31.

Mouse model of N-acetylgalactosamine-6-sulfate sulfatase deficiency (Galns-/-) produced by targeted disruption of the gene defective in Morquio A disease.

Tomatsu S, Orii KO, Vogler C, Nakayama J, Levy B, Grubb JH, Gutierrez MA, Shim S, Yamaguchi S, Nishioka T, Montano AM, Noguchi A, Orii T, Kondo N, Sly WS.

Hum Mol Genet. 2003 Dec 15;12(24):3349-58. Epub 2003 Oct 28.

PMID:
14583446
32.

Localization of carbonic anhydrase XII to the basolateral membrane of H+-secreting cells of mouse and rat kidney.

Kyllönen MS, Parkkila S, Rajaniemi H, Waheed A, Grubb JH, Shah GN, Sly WS, Kaunisto K.

J Histochem Cytochem. 2003 Sep;51(9):1217-24.

PMID:
12923247
33.

Production of MPS VII mouse (Gus(tm(hE540A x mE536A)Sly)) doubly tolerant to human and mouse beta-glucuronidase.

Tomatsu S, Orii KO, Vogler C, Grubb JH, Snella EM, Gutierrez M, Dieter T, Holden CC, Sukegawa K, Orii T, Kondo N, Sly WS.

Hum Mol Genet. 2003 May 1;12(9):961-73.

34.

Missense models [Gustm(E536A)Sly, Gustm(E536Q)Sly, and Gustm(L175F)Sly] of murine mucopolysaccharidosis type VII produced by targeted mutagenesis.

Tomatsu S, Orii KO, Vogler C, Grubb JH, Snella EM, Gutierrez MA, Dieter T, Sukegawa K, Orii T, Kondo N, Sly WS.

Proc Natl Acad Sci U S A. 2002 Nov 12;99(23):14982-7. Epub 2002 Oct 28.

35.

Regulation of transferrin-mediated iron uptake by HFE, the protein defective in hereditary hemochromatosis.

Waheed A, Grubb JH, Zhou XY, Tomatsu S, Fleming RE, Costaldi ME, Britton RS, Bacon BR, Sly WS.

Proc Natl Acad Sci U S A. 2002 Mar 5;99(5):3117-22. Epub 2002 Feb 26.

36.

Methylation patterns of the human beta-glucuronidase gene locus: boundaries of methylation and general implications for frequent point mutations at CpG dinucleotides.

Tomatsu S, Orii KO, Islam MR, Shah GN, Grubb JH, Sukegawa K, Suzuki Y, Orii T, Kondo N, Sly WS.

Genomics. 2002 Mar;79(3):363-75.

PMID:
11863366
37.

Biodistribution, kinetics, and efficacy of highly phosphorylated and non-phosphorylated beta-glucuronidase in the murine model of mucopolysaccharidosis VII.

Sands MS, Vogler CA, Ohlemiller KK, Roberts MS, Grubb JH, Levy B, Sly WS.

J Biol Chem. 2001 Nov 16;276(46):43160-5. Epub 2001 Sep 18.

38.

Crystal structure of the dimeric extracellular domain of human carbonic anhydrase XII, a bitopic membrane protein overexpressed in certain cancer tumor cells.

Whittington DA, Waheed A, Ulmasov B, Shah GN, Grubb JH, Sly WS, Christianson DW.

Proc Natl Acad Sci U S A. 2001 Aug 14;98(17):9545-50. Epub 2001 Aug 7.

39.

Active site mutant transgene confers tolerance to human beta-glucuronidase without affecting the phenotype of MPS VII mice.

Sly WS, Vogler C, Grubb JH, Zhou M, Jiang J, Zhou XY, Tomatsu S, Bi Y, Snella EM.

Proc Natl Acad Sci U S A. 2001 Feb 27;98(5):2205-10. Epub 2001 Feb 13.

40.

Expression of membrane-associated carbonic anhydrase XIV on neurons and axons in mouse and human brain.

Parkkila S, Parkkila AK, Rajaniemi H, Shah GN, Grubb JH, Waheed A, Sly WS.

Proc Natl Acad Sci U S A. 2001 Feb 13;98(4):1918-23.

41.

Purification and kinetic analysis of recombinant CA XII, a membrane carbonic anhydrase overexpressed in certain cancers.

Ulmasov B, Waheed A, Shah GN, Grubb JH, Sly WS, Tu C, Silverman DN.

Proc Natl Acad Sci U S A. 2000 Dec 19;97(26):14212-7.

42.

Mitochondrial carbonic anhydrase CA VB: differences in tissue distribution and pattern of evolution from those of CA VA suggest distinct physiological roles.

Shah GN, Hewett-Emmett D, Grubb JH, Migas MC, Fleming RE, Waheed A, Sly WS.

Proc Natl Acad Sci U S A. 2000 Feb 15;97(4):1677-82.

43.

Expression of a novel transmembrane carbonic anhydrase isozyme XII in normal human gut and colorectal tumors.

Kivelä A, Parkkila S, Saarnio J, Karttunen TJ, Kivelä J, Parkkila AK, Waheed A, Sly WS, Grubb JH, Shah G, Türeci O, Rajaniemi H.

Am J Pathol. 2000 Feb;156(2):577-84.

44.

Identification of carbonic anhydrase XII as the membrane isozyme expressed in the normal human endometrial epithelium.

Karhumaa P, Parkkila S, Türeci O, Waheed A, Grubb JH, Shah G, Parkkila A, Kaunisto K, Tapanainen J, Sly WS, Rajaniemi H.

Mol Hum Reprod. 2000 Jan;6(1):68-74.

PMID:
10611263
45.

Active site residues of human beta-glucuronidase. Evidence for Glu(540) as the nucleophile and Glu(451) as the acid-base residue.

Islam MR, Tomatsu S, Shah GN, Grubb JH, Jain S, Sly WS.

J Biol Chem. 1999 Aug 13;274(33):23451-5.

46.
47.

Enzyme replacement therapy improves reproductive performance in mucopolysaccharidosis type VII mice but does not prevent postnatal losses.

Soper BW, Pung AW, Vogler CA, Grubb JH, Sly WS, Barker JE.

Pediatr Res. 1999 Feb;45(2):180-6.

PMID:
10022587
48.

Identification of Glu-540 as the catalytic nucleophile of human beta-glucuronidase using electrospray mass spectrometry.

Wong AW, He S, Grubb JH, Sly WS, Withers SG.

J Biol Chem. 1998 Dec 18;273(51):34057-62.

49.

Human carbonic anhydrase XII: cDNA cloning, expression, and chromosomal localization of a carbonic anhydrase gene that is overexpressed in some renal cell cancers.

Türeci O, Sahin U, Vollmar E, Siemer S, Göttert E, Seitz G, Parkkila AK, Shah GN, Grubb JH, Pfreundschuh M, Sly WS.

Proc Natl Acad Sci U S A. 1998 Jun 23;95(13):7608-13.

50.

Structure of human beta-glucuronidase reveals candidate lysosomal targeting and active-site motifs.

Jain S, Drendel WB, Chen ZW, Mathews FS, Sly WS, Grubb JH.

Nat Struct Biol. 1996 Apr;3(4):375-81.

PMID:
8599764

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