L-iduronidase in cultured human fibroblasts and liver

Reuben Matalon; J. A. Cifonelli; Albert Dorfman

doi:10.1016/0006-291X(71)90108-2

L-iduronidase in cultured human fibroblasts and liver

Reuben Matalon, J. A. Cifonelli, Albert Dorfman

Research output: Contribution to journal › Article › peer-review

46 Scopus citations

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 R_f of iduronolactone on paper chromatography.

Original language	English (US)
Pages (from-to)	340-345
Number of pages	6
Journal	Biochemical and Biophysical Research Communications
Volume	42
Issue number	2
DOIs	https://doi.org/10.1016/0006-291X(71)90108-2
State	Published - Jan 22 1971
Externally published	Yes

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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10.1016/0006-291X(71)90108-2

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title = "L-iduronidase in cultured human fibroblasts and liver",

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.",

author = "Reuben Matalon and Cifonelli, {J. A.} and Albert Dorfman",

note = "Funding Information: This investigation was supported by PHS Res. Grant #AM-O5996 from the NIAMD, PHS Res. Grant #HD-04583 from the NICHD, and by a Res. Grant from the National Cystic Fibrosis Foundation.",

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doi = "10.1016/0006-291X(71)90108-2",

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T1 - L-iduronidase in cultured human fibroblasts and liver

AU - Matalon, Reuben

AU - Cifonelli, J. A.

AU - Dorfman, Albert

N1 - Funding Information: This investigation was supported by PHS Res. Grant #AM-O5996 from the NIAMD, PHS Res. Grant #HD-04583 from the NICHD, and by a Res. Grant from the National Cystic Fibrosis Foundation.

PY - 1971/1/22

Y1 - 1971/1/22

N2 - 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.

AB - 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.

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L-iduronidase in cultured human fibroblasts and liver

Abstract

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