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Literature DB >> 200837

Glycosphingolipid hydrolases: properties and molecular genetics.

M Wan Ho, A G Norden, J A Alhadeff, J S O'Brien.

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.

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Year: 1977 PMID： 200837 DOI： 10.1007/bf01730832

Source DB: PubMed Journal: Mol Cell Biochem ISSN： 0300-8177 Impact factor: 3.396

151 in total

1. The hydrolysis of Tay-Sachs ganglioside (TSG) by human N-acetyl-beta-D-hexosaminidase A.

Authors: K Sandhoff
Journal: FEBS Lett Date: 1970-12-18 Impact factor: 4.124

2. Polymorphism of human alpha fucosidase.

Authors: B M Turner; V S Turner; N G Beratis; K Hirschhorn
Journal: Am J Hum Genet Date: 1975-09 Impact factor: 11.025

3. Characterization of human alpha-galactosidase A and B before and after neuraminidase treatment.

Authors: G Romeo; G Di Matteo; M D'urso; S C Li; Y T Li
Journal: Biochim Biophys Acta Date: 1975-06-24

4. Ganglioside catabolism in hexosaminidase A-deficient adults.

Authors: J F Tallman; R O Brady; R Navon; B Padeh
Journal: Nature Date: 1974-11-15 Impact factor: 49.962

5. Isolation and characterization of glucocerebrosidase from human placental tissue.

Authors: P G Pentchev; R O Brady; S R Hibbert; A E Gal; D Shapiro
Journal: J Biol Chem Date: 1973-08-10 Impact factor: 5.157

6. Anomeric structure of globoside and ceramide grihexoside of human erythrocytes and hamster fibroblasts.

Authors: S I Hakomori; B Siddiqui; Y T Li; S C Li; C G Hellerqvist
Journal: J Biol Chem Date: 1971-04-10 Impact factor: 5.157

7. The synthesis of complex carbohydrates by multiglycosyltransferase systems and their potential function in intercellular adhesion.

Authors: S Roseman
Journal: Chem Phys Lipids Date: 1970-10 Impact factor: 3.329

Glycosphingolipid hydrolases: properties and molecular genetics.

1. The hydrolysis of Tay-Sachs ganglioside (TSG) by human N-acetyl-beta-D-hexosaminidase A.

2. Polymorphism of human alpha fucosidase.

3. Characterization of human alpha-galactosidase A and B before and after neuraminidase treatment.

4. Ganglioside catabolism in hexosaminidase A-deficient adults.

5. Isolation and characterization of glucocerebrosidase from human placental tissue.

6. Anomeric structure of globoside and ceramide grihexoside of human erythrocytes and hamster fibroblasts.

7. The synthesis of complex carbohydrates by multiglycosyltransferase systems and their potential function in intercellular adhesion.

8. Animal models of human ganglioside storage diseases.

9. Nature of the mutation in adult beta-galactosidase deficient patients.

10. Isolation and characterization of glucosylsphingosine from Gaucher's spleen.

Review 1. Biochemistry and genetics of gangliosidoses.

2. Ceramide metabolism in brain.