87 related articles for article (PubMed ID: 6817758)
1. Morquio disease, type B: activation of GM1-beta-galactosidase by GM1-activator protein.
Paschke E; Kresse H
Biochem Biophys Res Commun; 1982 Nov; 109(2):568-75. PubMed ID: 6817758
[No Abstract] [Full Text] [Related]
2. Hydrolysis of lactosylceramide by human galactosylceramidase and GM1-beta-galactosidase in a detergent-free system and its stimulation by sphingolipid activator proteins, sap-B and sap-C. Activator proteins stimulate lactosylceramide hydrolysis.
Zschoche A; Fürst W; Schwarzmann G; Sanhoff K
Eur J Biochem; 1994 May; 222(1):83-90. PubMed ID: 8200356
[TBL] [Abstract][Full Text] [Related]
3. Properties of a protein activator of glycosphingolipid hydrolysis isolated from the liver of a patient with GM1 gangliosidosis, type 1.
Inui K; Wenger DA
Biochem Biophys Res Commun; 1982 Mar; 105(2):745-51. PubMed ID: 6807306
[No Abstract] [Full Text] [Related]
4. Specificity of galactosylceramidase activation by phosphatidylserine.
Hanada E; Suzuki K
Biochim Biophys Acta; 1980 Aug; 619(2):396-402. PubMed ID: 6773584
[TBL] [Abstract][Full Text] [Related]
5. Molecular basis of GM1 gangliosidosis and Morquio disease, type B. Structure-function studies of lysosomal beta-galactosidase and the non-lysosomal beta-galactosidase-like protein.
Callahan JW
Biochim Biophys Acta; 1999 Oct; 1455(2-3):85-103. PubMed ID: 10571006
[TBL] [Abstract][Full Text] [Related]
6. Morquio B syndrome: a primary defect in beta-galactosidase.
van der Horst GT; Kleijer WJ; Hoogeveen AT; Huijmans JG; Blom W; van Diggelen OP
Am J Med Genet; 1983 Oct; 16(2):261-75. PubMed ID: 6418007
[TBL] [Abstract][Full Text] [Related]
7. Processing of human beta-galactosidase in GM1-gangliosidosis and Morquio B syndrome.
Hoogeveen AT; Graham-Kawashima H; d'Azzo A; Galjaard H
J Biol Chem; 1984 Feb; 259(3):1974-7. PubMed ID: 6420416
[TBL] [Abstract][Full Text] [Related]
8. Spondyloepiphyseal dysplasia, corneal clouding, normal intelligence and acid beta-galactosidase deficiency.
O'Brien JS; Gugler E; Giedion A; Wiessmann U; Herschkowitz N; Meier C; Leroy J
Clin Genet; 1976 May; 9(5):495-504. PubMed ID: 817853
[TBL] [Abstract][Full Text] [Related]
9. [Use of fluorescence-labelled cerebroside as a substrate for galactosylceramidase of human skin fibroblasts].
Semeniuk EP; Vidershaĭn GIa
Biokhimiia; 1984 Sep; 49(9):1556-60. PubMed ID: 6549145
[TBL] [Abstract][Full Text] [Related]
10. An activator protein of oligosaccharide sialidase.
Chabás A; Guardiola A; Burguera JM
Biochem Int; 1987 Aug; 15(2):449-57. PubMed ID: 3124850
[TBL] [Abstract][Full Text] [Related]
11. A microassay for acid beta-galactosidase activity toward asialofetuin.
Mutoh T; Naoi M; Sobue I; Kiuchi K; Nagatsu T
Clin Chim Acta; 1985 Nov; 152(3):307-14. PubMed ID: 2415273
[TBL] [Abstract][Full Text] [Related]
12. Molecular form and subcellular distribution of acid beta-galactosidase in fibroblasts from patients with GM1 gangliosidosis, Morquio B disease and galactosialidosis.
Takiyama N; Itoh K; Shimmoto M; Nishimoto J; Inui K; Sakuraba H; Suzuki Y
Brain Dev; 1997 Mar; 19(2):126-30. PubMed ID: 9105659
[TBL] [Abstract][Full Text] [Related]
13. A protein activator of galactosylceramide beta-galactosidase.
Wenger DA; Sattler M; Roth S
Biochim Biophys Acta; 1982 Sep; 712(3):639-49. PubMed ID: 7126630
[TBL] [Abstract][Full Text] [Related]
14. Kinetic properties of beta-galactosidase in Morquio B disease.
Czartoryska B; Gorska D; Abramowicz T
Acta Anthropogenet; 1983; 7(4):301-7. PubMed ID: 6439230
[TBL] [Abstract][Full Text] [Related]
15. Isoelectric focusing of galactosylceramide beta-galactosylceramide beta-galactosidase in cultured skin fibroblasts of patients with Krabbe's globoid-cell leucodystrophy.
Besley GT
Biochem Soc Trans; 1975; 3(2):241-4. PubMed ID: 1132549
[No Abstract] [Full Text] [Related]
16. Biosynthesis of the sulfatide/GM1 activator protein (SAP-1) in control and mutant cultured skin fibroblasts.
Fujibayashi S; Wenger DA
Biochim Biophys Acta; 1986 Feb; 875(3):554-62. PubMed ID: 3081038
[TBL] [Abstract][Full Text] [Related]
17. Saposins (sap) A and C activate the degradation of galactosylsphingosine.
Harzer K; Hiraiwa M; Paton BC
FEBS Lett; 2001 Nov; 508(1):107-10. PubMed ID: 11707278
[TBL] [Abstract][Full Text] [Related]
18. Biochemical, immunological, and structural studies on a sphingolipid activator protein (SAP-1).
Inui K; Wenger DA
Arch Biochem Biophys; 1984 Sep; 233(2):556-64. PubMed ID: 6435528
[TBL] [Abstract][Full Text] [Related]
19. A case of GM1-gangliosidosis type I: glycosphingolipid profiles of urine and transformed lymphocytes and beta-D-galactosidase activities in peripheral lymphocytes, cultured skin fibroblasts and transformed lymphocytes.
Asano K; Shindo N; Nakasuji M; Inamori K; Ohta M; Matsushita T; Yamaguchi M; Oshima M
Jpn J Exp Med; 1990 Apr; 60(2):73-9. PubMed ID: 2117086
[TBL] [Abstract][Full Text] [Related]
20. Novel beta-galactosidase gene mutation p.W273R in a woman with mucopolysaccharidosis type IVB (Morquio B) and lack of response to in vitro chaperone treatment of her skin fibroblasts.
Gucev ZS; Tasic V; Jancevska A; Zafirovski G; Kremensky I; Sinigerska I; Nanba E; Higaki K; Gucev F; Suzuki Y
Am J Med Genet A; 2008 Jul; 146A(13):1736-40. PubMed ID: 18546276
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]