221 related articles for article (PubMed ID: 1824846)
1. 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]
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. 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]
4. 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]
5. GM2-ganglioside metabolism in hexosaminidase A deficiency states: determination in situ using labeled GM2 added to fibroblast cultures.
Raghavan SS; Krusell A; Krusell J; Lyerla TA; Kolodny EH
Am J Hum Genet; 1985 Nov; 37(6):1071-82. PubMed ID: 2934978
[TBL] [Abstract][Full Text] [Related]
6. 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]
7. 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]
8. 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]
9. Membrane lipids and their degradation compounds control GM2 catabolism at intralysosomal luminal vesicles.
Anheuser S; Breiden B; Sandhoff K
J Lipid Res; 2019 Jun; 60(6):1099-1111. PubMed ID: 30988135
[TBL] [Abstract][Full Text] [Related]
10. Specific recognition of N-acetylneuraminic acid in the GM2 epitope by human GM2 activator protein.
Li SC; Wu YY; Sugiyama E; Taki T; Kasama T; Casellato R; Sonnino S; Li YT
J Biol Chem; 1995 Oct; 270(41):24246-51. PubMed ID: 7592631
[TBL] [Abstract][Full Text] [Related]
11. 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]
12. The protein activator specific for the enzymic hydrolysis of GM2 ganglioside in normal human brain and brains of three types of GM2 gangliosidosis.
Hirabayashi Y; Li YT; Li SC
J Neurochem; 1983 Jan; 40(1):168-75. PubMed ID: 6848657
[TBL] [Abstract][Full Text] [Related]
13. 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]
14. 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]
15. 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]
16. Ganglioside loading of cultured fibroblasts: a provocative method for the diagnosis of the GM2 gangliosidoses.
Charrow J; Binns HJ
Clin Chim Acta; 1986 Apr; 156(1):41-9. PubMed ID: 2938852
[TBL] [Abstract][Full Text] [Related]
17. The enzyme-binding region of human GM2-activator protein.
Wendeler M; Werth N; Maier T; Schwarzmann G; Kolter T; Schoeniger M; Hoffmann D; Lemm T; Saenger W; Sandhoff K
FEBS J; 2006 Mar; 273(5):982-91. PubMed ID: 16478472
[TBL] [Abstract][Full Text] [Related]
18. 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]
19. 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; 260(12):7568-72. PubMed ID: 3158659
[TBL] [Abstract][Full Text] [Related]
20. Plasma G
Blondel A; Kraoua I; Marcelino C; Khrouf W; Schlemmer D; Ganne B; Caillaud C; Fernández-Eulate G; Turki IBY; Dauriat B; Bonnefont-Rousselot D; Nadjar Y; Lamari F
Mol Genet Metab; 2023 Feb; 138(2):106983. PubMed ID: 36709536
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
