Literature DB >> 239890

The activator of cerebroside sulphatase. Purification from human liver and identification as a protein.

G Fischer, H Jatzkewitz.   

Abstract

1) A heat-stable activator of human sulphatase A (cerebroside sulphatase) was purified from human liver. It is required for the enzymatic degradation of cerebroside sulphates (sulphatides) in buffers (ionic strength greater than or equal 0.2) with osmolarity in the physiological range. 2) The purification steps involve extraction, acetone precipitation, heat treatment, isoelectric focusing and gel filtration. 3) Based on the definition of a specific activator unit, the purification of the final preparation was approximately 2000-fold over the acetone precipitation and several thousand-fold in the overall procedure. 4) The purified activator migrated as a single protein band when subjected to gel electrophoresis. Its effect was abolished after treatement with pronase E. The apparent molecular weight as determined by gel filtration was 21 500 +/- 1500; the isoelectric point was 4.3. 5) The activating effect of this protein factor and of taurodeoxycholate on cerebroside sulphatase activity was compared on a weight and molar basis.

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Year:  1975        PMID: 239890     DOI: 10.1515/bchm2.1975.356.1.605

Source DB:  PubMed          Journal:  Hoppe Seylers Z Physiol Chem        ISSN: 0018-4888


  26 in total

Review 1.  Basic findings and current developments in sphingolipidoses.

Authors:  H Pilz; R Heipertz; D Seidel
Journal:  Hum Genet       Date:  1979-03-12       Impact factor: 4.132

2.  Late-onset Krabbe disease initially diagnosed as cerebroside sulfatase activator deficiency.

Authors:  A L Fluharty; L Neidengard; D Holtzman; H Kihara
Journal:  Metab Brain Dis       Date:  1986-09       Impact factor: 3.584

Review 3.  Glycosphingolipid hydrolases: properties and molecular genetics.

Authors:  M Wan Ho; A G Norden; J A Alhadeff; J S O'Brien
Journal:  Mol Cell Biochem       Date:  1977-10-07       Impact factor: 3.396

4.  Inheritance of metachromatic leukodystrophy.

Authors:  U Langenbeck; P Dunker; R Heipertz; H Pilz
Journal:  Am J Hum Genet       Date:  1977-11       Impact factor: 11.025

5.  Placental steroid deficiency: association with arylsulfatase A deficiency.

Authors:  J Vidgoff; M M Buxman; L J Shapiro; R L Dimond; T G Wilson; C A Hepburn; T Tabei; W R Heinrichs
Journal:  Am J Hum Genet       Date:  1982-05       Impact factor: 11.025

6.  The AB-variant of metachromatic leukodystrophy (postulated activator protein deficiency). Light and electron microscopic findings in a sural nerve biopsy.

Authors:  A F Hahn; B A Gordon; J J Gilbert; G G Hinton
Journal:  Acta Neuropathol       Date:  1981       Impact factor: 17.088

7.  [Chance and discovery in research on the cause of infantile amaurotic idiocy].

Authors:  H Jatzkewitz
Journal:  Naturwissenschaften       Date:  1981-05

8.  Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy.

Authors:  R L Stevens; A L Fluharty; H Kihara; M M Kaback; L J Shapiro; B Marsh; K Sandhoff; G Fischer
Journal:  Am J Hum Genet       Date:  1981-11       Impact factor: 11.025

9.  Ganglioside GM2 N-acetyl-beta-D-galactosaminidase activity in cultured fibroblasts of late-infantile and adult GM2 gangliosidosis patients and of healthy probands with low hexosaminidase level.

Authors:  E Conzelmann; H J Kytzia; R Navon; K Sandhoff
Journal:  Am J Hum Genet       Date:  1983-09       Impact factor: 11.025

10.  The AB-variant of GM2-gangliosidosis. Clinical, biochemical, and pathological studies of two patients.

Authors:  J E Goldman; T Yamanaka; I Rapin; M Adachi; K Suzuki; K Suzuki
Journal:  Acta Neuropathol       Date:  1980       Impact factor: 17.088

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