Document Detail

Complementation of multiple sulfatase deficiency in somatic cell hybrids.
MedLine Citation:
PMID:  6731437     Owner:  NLM     Status:  MEDLINE    
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.
K Fedde; A L Horwitz
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of human genetics     Volume:  36     ISSN:  0002-9297     ISO Abbreviation:  Am. J. Hum. Genet.     Publication Date:  1984 May 
Date Detail:
Created Date:  1984-07-19     Completed Date:  1984-07-19     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0370475     Medline TA:  Am J Hum Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  623-33     Citation Subset:  IM    
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MeSH Terms
Electrophoresis, Agar Gel
Fibroblasts / enzymology
Genetic Complementation Test
Hybrid Cells / enzymology*
Metabolism, Inborn Errors / enzymology,  genetics*
Sulfatases / deficiency*,  genetics
Grant Support
Reg. No./Substance:
EC 3.1.6.-/Sulfatases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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