Literature DB >> 4262638

Simulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium.

S O Lie, V A McKusick, E F Neufeld.   

Abstract

Catabolism of sulfated mucopolysaccharide by normal human fibroblasts in culture is progressively inhibited as the pH of the growth medium is raised from 6.8 to 8.0. The final cell density increases with the change in pH. The capacity to degrade mucopolysaccharide is rapidly restored by lowering the pH, and this reactivation does not require protein synthesis. Such pH dependence is not observed in cells from patients with genetic impairment of mucopolysaccharide degradation, such as the Hurler or Hunter syndromes. These results may have relevance not only to studies of mucopolysaccharide metabolism in cell culture, but also to the use of metachromasia as a genetic marker and to the observation that normal fibroblasts are released from contact inhibition of growth as the pH of the growth medium is raised.

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Year:  1972        PMID: 4262638      PMCID: PMC426940          DOI: 10.1073/pnas.69.9.2361

Source DB:  PubMed          Journal:  Proc Natl Acad Sci U S A        ISSN: 0027-8424            Impact factor:   11.205


  24 in total

1.  The defect in Hurler's and Hunter's syndromes: faulty degradation of mucopolysaccharide.

Authors:  J C Fratantoni; C W Hall; E F Neufeld
Journal:  Proc Natl Acad Sci U S A       Date:  1968-06       Impact factor: 11.205

2.  Cellular metachromasia, a genetic marker for studying the mucopolysaccharidoses.

Authors:  B S Danes; A G Bearn
Journal:  Lancet       Date:  1967-02-04       Impact factor: 79.321

3.  Acid mucopolysaccharides in cultured human fibroblasts.

Authors:  R Matalon; A Dorfman
Journal:  Lancet       Date:  1969-10-18       Impact factor: 79.321

4.  Cultivated amniotic-fluid cells and fibroblasts derived from families with cystic fibrosis.

Authors:  H L Nadler; J M Wodnicki; M A Swae; M E O'Flynn
Journal:  Lancet       Date:  1969-07-12       Impact factor: 79.321

5.  Hurler's syndrome: demonstration of an inherited disorder of connective tissue in cell culture.

Authors:  B S Danes; A G Bearn
Journal:  Science       Date:  1965-08-27       Impact factor: 47.728

6.  Myotonic muscular dystrophy: abnormalities in fibroblast culture.

Authors:  M R Swift; M J Finegold
Journal:  Science       Date:  1969-07-18       Impact factor: 47.728

7.  Gaucher's disease: a genetic disease detected in skin fibroblast cultures.

Authors:  B S Danes; A G Bearn
Journal:  Science       Date:  1968-09-27       Impact factor: 47.728

8.  Glycolipid and mucopolysaccharide abnormality in fibroblasts of fabry's disease.

Authors:  R Matalon; A Dorfman; G Dawson; C C Sweeley
Journal:  Science       Date:  1969-06-27       Impact factor: 47.728

9.  Cystic fibrosis of the pancreas. A study in cell culture.

Authors:  B S Danes; A G Bearn
Journal:  J Exp Med       Date:  1969-04-01       Impact factor: 14.307

10.  Hurler's syndrome. A genetic study in cell culture.

Authors:  B S Danes; A G Bearn
Journal:  J Exp Med       Date:  1966-01-01       Impact factor: 14.307

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  17 in total

1.  Supravalvular aortic stenosis-infantile hypercalcaemia syndrome: in vitro hypersensitivity to vitamin D2 and calcium.

Authors:  D M Becroft; D Chambers
Journal:  J Med Genet       Date:  1976-06       Impact factor: 6.318

2.  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

3.  Lysosomal composition in cultured vascular smooth muscle cells: electron probe analysis.

Authors:  M R James-Kracke; B F Sloane; H Shuman; A P Somlyo
Journal:  Proc Natl Acad Sci U S A       Date:  1979-12       Impact factor: 11.205

Review 4.  The cornea--structure and macromolecules in health and disease. A review.

Authors:  G K Klintworth
Journal:  Am J Pathol       Date:  1977-12       Impact factor: 4.307

5.  Development of an established cell line derived from Dasypus novemcinctus (armadillo), a laboratory animal susceptible to infection by Mycobacterium leprae.

Authors:  R L Amborski; G LoPiccolo; G F Amborski
Journal:  Experientia       Date:  1974-05-15

Review 6.  Mucopolysaccharidoses and mucolipidoses.

Authors:  F Van Hoof
Journal:  J Clin Pathol Suppl (R Coll Pathol)       Date:  1974

7.  Cystic fibrosis: demonstration of an abnormality in mucopolysaccharides in cultured lymphoid lines.

Authors:  B S Danes; J E Backofen; B K Rottell
Journal:  Biochem Genet       Date:  1974-11       Impact factor: 1.890

8.  Reversible change in the fibroblast lysosomal enzyme dipeptidyl aminopeptidase-1 (cathepsin C) related to the commercial source of fetal bovine serum in the culture medium.

Authors:  M J Doughty; M H Davis; E Gruenstein
Journal:  In Vitro Cell Dev Biol       Date:  1985-06

9.  Endocytosis of sulphated proteoglycans by cultured skin fibroblasts.

Authors:  R Prinz; J Schwermann; E Buddecke; K von Figura
Journal:  Biochem J       Date:  1978-12-15       Impact factor: 3.857

10.  Increased glycosaminoglycan accumulation as a genetic characteristic in cell cultures of one variety of dominant dystrophic epidermolysis bullosa.

Authors:  E A Bauer; W K Fiehler; N B Esterly
Journal:  J Clin Invest       Date:  1979-07       Impact factor: 14.808

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