Glycosphingolipid hydrolases: properties and molecular genetics
- PMID: 200837
- DOI: 10.1007/BF01730832
Glycosphingolipid hydrolases: properties and molecular genetics
Abstract
This is a review of the properties and molecular genetics of six lysosomal hydrolases: beta-galactosidase, hexosaminidases A and B, alpha-galactosidase, beta-glucosidase and alpha-fucosidase. Each enzyme is discussed with regards to isoenzymes and substrate specificity, subunit structure, genetic relationship of isoenzymes and genetic variants. The molecular genetics of human diseases caused by deficiencies of each enzyme are discussed.
Similar articles
-
Cloning and characterization of a novel cold-active glycoside hydrolase family 1 enzyme with β-glucosidase, β-fucosidase and β-galactosidase activities.BMC Biotechnol. 2013 Mar 15;13:22. doi: 10.1186/1472-6750-13-22. BMC Biotechnol. 2013. PMID: 23497058 Free PMC article.
-
Purification of glycoside hydrolases from Bacteroides fragilis.Appl Environ Microbiol. 1980 Jul;40(1):40-7. doi: 10.1128/aem.40.1.40-47.1980. Appl Environ Microbiol. 1980. PMID: 6250477 Free PMC article.
-
[Total activity and distribution of multiple forms of lysosomal glycosidases in subfractions of human leukocytes].Vopr Med Khim. 1984 Sep-Oct;30(5):76-81. Vopr Med Khim. 1984. PMID: 6241372 Russian.
-
Biochemistry of the multiple forms of glycosidases in plants.Adv Enzymol Relat Areas Mol Biol. 1984;56:141-249. doi: 10.1002/9780470123027.ch3. Adv Enzymol Relat Areas Mol Biol. 1984. PMID: 6320603 Review. No abstract available.
-
Design and synthesis of 5a-carbaglycopyranosylamime glycosidase inhibitors.Curr Top Med Chem. 2009;9(1):58-75. doi: 10.2174/156802609787354324. Curr Top Med Chem. 2009. PMID: 19199996 Review.
Cited by
-
Biochemistry and genetics of gangliosidoses.Hum Genet. 1979;50(2):107-43. doi: 10.1007/BF00390234. Hum Genet. 1979. PMID: 116955 Review.
-
Ceramide metabolism in brain.Mol Cell Biochem. 1979 Jan 15;23(1):17-25. doi: 10.1007/BF00226676. Mol Cell Biochem. 1979. PMID: 423891