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Journal Abstract Search

256 related items for PubMed ID: 12527415

  • 1. A single site in human beta-hexosaminidase A binds both 6-sulfate-groups on hexosamines and the sialic acid moiety of GM2 ganglioside.
    Sharma R, Bukovac S, Callahan J, Mahuran D.
    Biochim Biophys Acta; 2003 Jan 20; 1637(1):113-8. PubMed ID: 12527415
    [Abstract] [Full Text] [Related]

  • 2. Identification of the 6-sulfate binding site unique to alpha-subunit-containing isozymes of human beta-hexosaminidase.
    Sharma R, Deng H, Leung A, Mahuran D.
    Biochemistry; 2001 May 08; 40(18):5440-6. PubMed ID: 11331008
    [Abstract] [Full Text] [Related]

  • 3. Identification of domains in human beta-hexosaminidase that determine substrate specificity.
    Pennybacker M, Liessem B, Moczall H, Tifft CJ, Sandhoff K, Proia RL.
    J Biol Chem; 1996 Jul 19; 271(29):17377-82. PubMed ID: 8663217
    [Abstract] [Full Text] [Related]

  • 4. A Pro504 --> Ser substitution in the beta-subunit of beta-hexosaminidase A inhibits alpha-subunit hydrolysis of GM2 ganglioside, resulting in chronic Sandhoff disease.
    Hou Y, McInnes B, Hinek A, Karpati G, Mahuran D.
    J Biol Chem; 1998 Aug 14; 273(33):21386-92. PubMed ID: 9694901
    [Abstract] [Full Text] [Related]

  • 5. Direct determination of the substrate specificity of the alpha-active site in heterodimeric beta-hexosaminidase A.
    Hou Y, Tse R, Mahuran DJ.
    Biochemistry; 1996 Apr 02; 35(13):3963-9. PubMed ID: 8672428
    [Abstract] [Full Text] [Related]

  • 6. Identification of an active acidic residue in the catalytic site of beta-hexosaminidase.
    Tse R, Vavougios G, Hou Y, Mahuran DJ.
    Biochemistry; 1996 Jun 11; 35(23):7599-607. PubMed ID: 8652542
    [Abstract] [Full Text] [Related]

  • 7. Specificity of mouse GM2 activator protein and beta-N-acetylhexosaminidases A and B. Similarities and differences with their human counterparts in the catabolism of GM2.
    Yuziuk JA, Bertoni C, Beccari T, Orlacchio A, Wu YY, Li SC, Li YT.
    J Biol Chem; 1998 Jan 02; 273(1):66-72. PubMed ID: 9417048
    [Abstract] [Full Text] [Related]

  • 8. The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A.
    Meier EM, Schwarzmann G, Fürst W, Sandhoff K.
    J Biol Chem; 1991 Jan 25; 266(3):1879-87. PubMed ID: 1824846
    [Abstract] [Full Text] [Related]

  • 9. Formation of a ternary complex between GM2 activator protein, GM2 ganglioside and hexosaminidase A.
    Yadao F, Hechtman P, Kaplan F.
    Biochim Biophys Acta; 1997 Jun 20; 1340(1):45-52. PubMed ID: 9217013
    [Abstract] [Full Text] [Related]

  • 10. In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside.
    Sinici I, Yonekawa S, Tkachyova I, Gray SJ, Samulski RJ, Wakarchuk W, Mark BL, Mahuran DJ.
    PLoS One; 2013 Jun 20; 8(3):e57908. PubMed ID: 23483939
    [Abstract] [Full Text] [Related]

  • 11. Identification of functional domains within the alpha and beta subunits of beta-hexosaminidase A through the expression of alpha-beta fusion proteins.
    Tse R, Wu YJ, Vavougios G, Hou Y, Hinek A, Mahuran DJ.
    Biochemistry; 1996 Aug 20; 35(33):10894-903. PubMed ID: 8718882
    [Abstract] [Full Text] [Related]

  • 12. Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis.
    Lemieux MJ, Mark BL, Cherney MM, Withers SG, Mahuran DJ, James MN.
    J Mol Biol; 2006 Jun 16; 359(4):913-29. PubMed ID: 16698036
    [Abstract] [Full Text] [Related]

  • 13. Inefficiency in GM2 ganglioside elimination by human lysosomal beta-hexosaminidase beta-subunit gene transfer to fibroblastic cell line derived from Sandhoff disease model mice.
    Itakura T, Kuroki A, Ishibashi Y, Tsuji D, Kawashita E, Higashine Y, Sakuraba H, Yamanaka S, Itoh K.
    Biol Pharm Bull; 2006 Aug 16; 29(8):1564-9. PubMed ID: 16880605
    [Abstract] [Full Text] [Related]

  • 14. Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A.
    Kytzia HJ, Sandhoff K.
    J Biol Chem; 1985 Jun 25; 260(12):7568-72. PubMed ID: 3158659
    [Abstract] [Full Text] [Related]

  • 15. Identification of candidate active site residues in lysosomal beta-hexosaminidase A.
    Fernandes MJ, Yew S, Leclerc D, Henrissat B, Vorgias CE, Gravel RA, Hechtman P, Kaplan F.
    J Biol Chem; 1997 Jan 10; 272(2):814-20. PubMed ID: 8995368
    [Abstract] [Full Text] [Related]

  • 16. The specificity of human N-acetyl-beta-D-hexosaminidases towards glycosphincolipids is determined by an activator protein.
    Conzelmann E, Sandhoff K.
    Adv Exp Med Biol; 1980 Jan 10; 125():295-306. PubMed ID: 6444774
    [Abstract] [Full Text] [Related]

  • 17. Substrate binding properties of the human liver hexosaminidase A activator protein.
    Hechtman P, Isaacs C, Smith-Jones L.
    Can J Biochem Cell Biol; 1985 Aug 10; 63(8):830-8. PubMed ID: 2933135
    [Abstract] [Full Text] [Related]

  • 18. Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease.
    Mark BL, Mahuran DJ, Cherney MM, Zhao D, Knapp S, James MN.
    J Mol Biol; 2003 Apr 11; 327(5):1093-109. PubMed ID: 12662933
    [Abstract] [Full Text] [Related]

  • 19. An alpha-subunit loop structure is required for GM2 activator protein binding by beta-hexosaminidase A.
    Zarghooni M, Bukovac S, Tropak M, Callahan J, Mahuran D.
    Biochem Biophys Res Commun; 2004 Nov 19; 324(3):1048-52. PubMed ID: 15485660
    [Abstract] [Full Text] [Related]

  • 20. [Recent advances in molecular genetics of GM2 gangliosidosis].
    Wakamatsu N.
    Nihon Rinsho; 1995 Dec 19; 53(12):2988-93. PubMed ID: 8577047
    [Abstract] [Full Text] [Related]

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