L-iduronidase in cultured human fibroblasts and liver

Reuben Matalon, J. A. Cifonelli, Albert Dorfman

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

Extracts of normal human livers and skin fibroblasts cultured from normal individuals and patients with the Hurler syndrome released L-iduronic acid when incubated with desulfated dermatan sulfate. Enzyme extracts of normal fibroblasts liberated larger amounts of L-induronic acid, as judged by paper chromatography, than did homogenates from Hurler fibroblasts. Preliminary studies with desulfated heparan sulfate using the same enzyme systems have also shown material with the Rf of iduronolactone on paper chromatography.

Original languageEnglish
Pages (from-to)340-345
Number of pages6
JournalBiochemical and Biophysical Research Communications
Volume42
Issue number2
DOIs
StatePublished - Jan 22 1971
Externally publishedYes

Fingerprint

Iduronidase
Fibroblasts
Liver
Paper Chromatography
Chromatography
Iduronic Acid
Mucopolysaccharidosis I
Dermatan Sulfate
Heparitin Sulfate
Enzymes
Skin
Acids

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

L-iduronidase in cultured human fibroblasts and liver. / Matalon, Reuben; Cifonelli, J. A.; Dorfman, Albert.

In: Biochemical and Biophysical Research Communications, Vol. 42, No. 2, 22.01.1971, p. 340-345.

Research output: Contribution to journalArticle

Matalon, Reuben ; Cifonelli, J. A. ; Dorfman, Albert. / L-iduronidase in cultured human fibroblasts and liver. In: Biochemical and Biophysical Research Communications. 1971 ; Vol. 42, No. 2. pp. 340-345.
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