Literature DB >> 4195824

Correction of celluar metachromasia in cultured fibroblasts in several inherited mucopolysaccharidoses.

B S Danes, A G Bearn.   

Abstract

Cultured fibroblasts from the genetic mucopolysaccharidoses store higher than normal amounts of the polyanionic glycosaminoglycans (mucopolysaccharides); histochemical staining with the cationic dyes toluidine blue O and Alcian blue detects such intracellular accumulation. With these stains as phenotypic markers, correction of the cellular abnormality by fibroblasts derived from different patients and their heterozygous parents has been observed among several genetic types of mucopolysaccharidoses. In this way, syndromes previously considered clinically homogeneous have been separated into distinct groups.

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Year:  1970        PMID: 4195824      PMCID: PMC283213          DOI: 10.1073/pnas.67.1.357

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


  15 in total

1.  Excretion of sulfated mucopolysaccharides in gargoylism (Hurler's syndrome).

Authors:  K MEYER; M M GRUMBACH; A LINKER; P HOFFMAN
Journal:  Proc Soc Exp Biol Med       Date:  1958-02

2.  Gargoylism; a mucopolysaccharidosis.

Authors:  G BRANTE
Journal:  Scand J Clin Lab Invest       Date:  1952       Impact factor: 1.713

3.  Differential staining of acid glycosaminoglycans (mucopolysaccharides) by alcian blue in salt solutions.

Authors:  J E Scott; J Dorling
Journal:  Histochemie       Date:  1965-10-01

Review 4.  The nosology of the mucopolysaccharidoses.

Authors:  V A McKusick
Journal:  Am J Med       Date:  1969-11       Impact factor: 4.965

5.  Acid mucopolysaccharides in cultured human fibroblasts.

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

6.  Diagnostic limitations of metachromasia.

Authors:  A Milunsky; J W Littlefield
Journal:  N Engl J Med       Date:  1969-11-13       Impact factor: 91.245

7.  A genetic study of cystic fibrosis of the pancreas in cell culture.

Authors:  A G Bearn; B S Danes
Journal:  Trans Assoc Am Physicians       Date:  1969

8.  Hurler's syndrome: a genetic study of clones in cell culture with particular reference to the Lyon hypothesis.

Authors:  B S Danes; A G Bearn
Journal:  J Exp Med       Date:  1967-09-01       Impact factor: 14.307

9.  Clonal growth of mammalian cells in vitro; growth characteristics of colonies from single HeLa cells with and without a feeder layer.

Authors:  T T PUCK; P I MARCUS; S J CIECIURA
Journal:  J Exp Med       Date:  1956-02-01       Impact factor: 14.307

10.  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

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  2 in total

1.  Serum dependency of cellular phenotype in mucopolysaccharidoses: the influence of autologous serum on metachromasia.

Authors:  L P ten Kate; G J Anders; C J de Groot
Journal:  Humangenetik       Date:  1973-03-23

2.  Defective function of renal lysosomes in mice with the Chediak-Higashi syndrome.

Authors:  D J Prieur; W C Davis; G A Padgett
Journal:  Am J Pathol       Date:  1972-05       Impact factor: 4.307

  2 in total

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