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Similar articles for PubMed (Select 9694901)

2.

Biochemical consequences of mutations causing the GM2 gangliosidoses.

Mahuran DJ.

Biochim Biophys Acta. 1999 Oct 8;1455(2-3):105-38. Review.

3.

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;29(8):1564-9.

4.

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;8(3):e57908. doi: 10.1371/journal.pone.0057908. Epub 2013 Mar 4.

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.

PMID:
8652542
7.
8.

The Val192Leu mutation in the alpha-subunit of beta-hexosaminidase A is not associated with the B1-variant form of Tay-Sachs disease.

Hou Y, Vavougios G, Hinek A, Wu KK, Hechtman P, Kaplan F, Mahuran DJ.

Am J Hum Genet. 1996 Jul;59(1):52-8.

9.

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.

PMID:
8718882
12.

Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases.

Huang JQ, Trasler JM, Igdoura S, Michaud J, Hanal N, Gravel RA.

Hum Mol Genet. 1997 Oct;6(11):1879-85.

13.

Metabolic correction in microglia derived from Sandhoff disease model mice.

Tsuji D, Kuroki A, Ishibashi Y, Itakura T, Itoh K.

J Neurochem. 2005 Sep;94(6):1631-8. Epub 2005 Aug 10.

PMID:
16092933
14.

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. Epub 2006 Apr 27.

15.

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.

16.

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.

17.

The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A.

Meier EM, Schwarzmann G, F├╝rst W, Sandhoff K.

J Biol Chem. 1991 Jan 25;266(3):1879-87.

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