205 related articles for article (PubMed ID: 9217013)
1. 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; 1340(1):45-52. PubMed ID: 9217013
[TBL] [Abstract][Full Text] [Related]
2. 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; 273(1):66-72. PubMed ID: 9417048
[TBL] [Abstract][Full Text] [Related]
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. PubMed ID: 16880605
[TBL] [Abstract][Full Text] [Related]
4. 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; 266(3):1879-87. PubMed ID: 1824846
[TBL] [Abstract][Full Text] [Related]
5. Biochemical characterization of the Cys138Arg substitution associated with the AB variant form of GM2 gangliosidosis: evidence that Cys138 is required for the recognition of the GM2 activator/GM2 ganglioside complex by beta-hexosaminidase A.
Xie B; Rigat B; Smiljanic-Georgijev N; Deng H; Mahuran D
Biochemistry; 1998 Jan; 37(3):814-21. PubMed ID: 9454570
[TBL] [Abstract][Full Text] [Related]
6. 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; 1637(1):113-8. PubMed ID: 12527415
[TBL] [Abstract][Full Text] [Related]
7. Degradation of membrane-bound ganglioside GM2 by beta -hexosaminidase A. Stimulation by GM2 activator protein and lysosomal lipids.
Werth N; Schuette CG; Wilkening G; Lemm T; Sandhoff K
J Biol Chem; 2001 Apr; 276(16):12685-90. PubMed ID: 11278374
[TBL] [Abstract][Full Text] [Related]
8. Expression and specificity of human GM2 activator protein.
Wu YY; Lockyer JM; Sugiyama E; Pavlova NV; Li YT; Li SC
J Biol Chem; 1994 Jun; 269(23):16276-83. PubMed ID: 8206933
[TBL] [Abstract][Full Text] [Related]
9. 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; 271(29):17377-82. PubMed ID: 8663217
[TBL] [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; 8(3):e57908. PubMed ID: 23483939
[TBL] [Abstract][Full Text] [Related]
11. 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; 63(8):830-8. PubMed ID: 2933135
[TBL] [Abstract][Full Text] [Related]
12. 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; 273(33):21386-92. PubMed ID: 9694901
[TBL] [Abstract][Full Text] [Related]
13. 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; 125():295-306. PubMed ID: 6444774
[TBL] [Abstract][Full Text] [Related]
14. 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; 35(13):3963-9. PubMed ID: 8672428
[TBL] [Abstract][Full Text] [Related]
15. Complexing of glycolipids and their transfer between membranes by the activator protein for degradation of lysosomal ganglioside GM2.
Conzelmann E; Burg J; Stephan G; Sandhoff K
Eur J Biochem; 1982 Apr; 123(2):455-64. PubMed ID: 6210531
[TBL] [Abstract][Full Text] [Related]
16. 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; 35(33):10894-903. PubMed ID: 8718882
[TBL] [Abstract][Full Text] [Related]
17. Juvenile GM2 gangliosidosis (AMB variant): inability to activate hexosaminidase A by activator protein.
Inui K; Grebner EE; Jackson LG; Wenger DA
Am J Hum Genet; 1983 Jul; 35(4):551-64. PubMed ID: 6224417
[TBL] [Abstract][Full Text] [Related]
18. 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. PubMed ID: 16092933
[TBL] [Abstract][Full Text] [Related]
19. Ganglioside GM2 levels in human melanoma cells: inverse correlation with lysosomal beta-hexosaminidase A activity.
Hoon DS; Kaback MM; Lim-Steele J; Tsuchida T; Morton DL; Irie RF
Biochem Int; 1992 Jul; 27(2):343-52. PubMed ID: 1386984
[TBL] [Abstract][Full Text] [Related]
20. Isolation and characterization of an activator protein for the hydrolysis of ganglioside GM2 from the roe of striped mullet (Mugil cephalus).
DeGasperi R; Li YT; Li SC
Biochem J; 1989 Jun; 260(3):777-83. PubMed ID: 2527497
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]