Literature DB >> 6731437

Complementation of multiple sulfatase deficiency in somatic cell hybrids.

K Fedde, A L Horwitz.   

Abstract

Multiple sulfatase deficiency (MSD) is an inherited disorder characterized by deficient activity of seven different sulfatases. Genetic complementation for steroid sulfatase (STS), arylsulfatase A, and N-acetylgalactosamine 6-SO4 sulfatase was demonstrated in somatic cell hybrids between MSD fibroblasts and mouse cells ( LA9 ) or Chinese hamster cells ( CHW ). In an electrophoretic system that separates human and rodent STS isozymes, enzyme from hybrids migrated as human enzyme. We concluded that the rodent cell complemented the MSD deficiency and allowed normal expression of the STS structural gene. Some MSD- LA9 hybrids showed significant levels of human arylsulfatase A activity, as shown by the immunoprecipitation of active enzyme by human-specific antiserum. Complementation was also suggested for N-acetylgalactosamine 6- sulfatate sulfatase (GalNAc-6S sulfatase) in several MSD- LA9 hybrids by the demonstration of a significant increase in activity (10-fold) over that of the GalNAc-6S sulfatase-deficient parental mouse and MSD cells. Thus, it was possible to demonstrate complementation for more than one sulfatase in a single MSD-rodent hybrid. Normal levels of sulfatase activity in hybrids indicate that the sulfatase structural genes are intact in MSD cells.

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Year:  1984        PMID: 6731437      PMCID: PMC1684451     

Source DB:  PubMed          Journal:  Am J Hum Genet        ISSN: 0002-9297            Impact factor:   11.025


  27 in total

1.  Ouabain as a selective agent in the isolation of somatic cell hybrids.

Authors:  R S Kucherlapati; R M Baker; F H Ruddle
Journal:  Birth Defects Orig Artic Ser       Date:  1975

2.  The nature of the residual arylsulfatase activity in metachromatic leukodystrophy.

Authors:  E Shapira; H L Nadler
Journal:  J Pediatr       Date:  1975-06       Impact factor: 4.406

Review 3.  Enzymic diagnosis of the genetic mucopolysaccharide storage disorders.

Authors:  C W Hall; I Liebaers; P Di Natale; E F Neufeld
Journal:  Methods Enzymol       Date:  1978       Impact factor: 1.600

4.  Effect of neuraminidase on the multiple forms of arylsulphatase B.

Authors:  A A Farooqui
Journal:  Biochimie       Date:  1976       Impact factor: 4.079

5.  Presence of arylsulfatase A (ARS A) in multiple sulfatase deficiency disorder fibroblasts.

Authors:  A L Fluharty; R L Stevens; L L Davis; L J Shapiro; H Kihara
Journal:  Am J Hum Genet       Date:  1978-05       Impact factor: 11.025

6.  Immunologic studies of arylsulfatase A in normal and metachromatic leukodystrophy liver.

Authors:  E Shapira; R R DeGregorio; H L Nadler
Journal:  Pediatr Res       Date:  1978-03       Impact factor: 3.756

7.  Multiple sulfatase deficiencies in cultured skin fibroblasts. Occurrence in patients with a variant form of metachromatic leukodystrophy.

Authors:  Y Eto; U N Wiesmann; J H Carson; N N Herschkowitz
Journal:  Arch Neurol       Date:  1974-02

8.  Studies in metachromatic leukodystrophy. XII. Multiple sulfatase deficiency.

Authors:  J H Austin
Journal:  Arch Neurol       Date:  1973-04

9.  A simplified method for cyanogen bromide activation of agarose for affinity chromatography.

Authors:  S C March; I Parikh; P Cuatrecasas
Journal:  Anal Biochem       Date:  1974-07       Impact factor: 3.365

10.  Improved techniques for the induction of mammalian cell hybridization by polyethylene glycol.

Authors:  R L Davidson; P S Gerald
Journal:  Somatic Cell Genet       Date:  1976-03
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  3 in total

1.  Multiple sulfatase deficiency with a novel biochemical presentation.

Authors:  G Constantopoulos
Journal:  Eur J Pediatr       Date:  1988-08       Impact factor: 3.183

2.  Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs.

Authors:  W Rommerskirch; K von Figura
Journal:  Proc Natl Acad Sci U S A       Date:  1992-04-01       Impact factor: 11.205

Review 3.  Multiple Sulfatase Deficiency: A Disease Comprising Mucopolysaccharidosis, Sphingolipidosis, and More Caused by a Defect in Posttranslational Modification.

Authors:  Lars Schlotawa; Laura A Adang; Karthikeyan Radhakrishnan; Rebecca C Ahrens-Nicklas
Journal:  Int J Mol Sci       Date:  2020-05-13       Impact factor: 5.923

  3 in total

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