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The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies.
Am J Hum Genet 1981; 33(4):576-83AJ

Abstract

The exogenous addition of iduronate sulfate sulfatase to cultured fibroblasts of Hunter patients resulted in a full correction of the metabolic defect as demonstrated by chemical and ultrastructural analyses. As little as 25% of the normal fibroblasts' enzyme levels were sufficient for this correction. The half-disappearance time of the internalized enzyme was 3-4 days. Prolonged incubation of corrected cells resulted in a gradual reaccumulation of mucopolysaccharides.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

6789675

Citation

Eliahu, R, et al. "The Correction of Hunter Fibroblasts By Exogenous Iduronate Sulfate Sulfatase: Biochemical and Ultrastructural Studies." American Journal of Human Genetics, vol. 33, no. 4, 1981, pp. 576-83.
Eliahu R, Sekeles E, Cohen R, et al. The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies. Am J Hum Genet. 1981;33(4):576-83.
Eliahu, R., Sekeles, E., Cohen, R., & Bach, G. (1981). The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies. American Journal of Human Genetics, 33(4), pp. 576-83.
Eliahu R, et al. The Correction of Hunter Fibroblasts By Exogenous Iduronate Sulfate Sulfatase: Biochemical and Ultrastructural Studies. Am J Hum Genet. 1981;33(4):576-83. PubMed PMID: 6789675.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The correction of Hunter fibroblasts by exogenous iduronate sulfate sulfatase: biochemical and ultrastructural studies. AU - Eliahu,R, AU - Sekeles,E, AU - Cohen,R, AU - Bach,G, PY - 1981/7/1/pubmed PY - 1981/7/1/medline PY - 1981/7/1/entrez SP - 576 EP - 83 JF - American journal of human genetics JO - Am. J. Hum. Genet. VL - 33 IS - 4 N2 - The exogenous addition of iduronate sulfate sulfatase to cultured fibroblasts of Hunter patients resulted in a full correction of the metabolic defect as demonstrated by chemical and ultrastructural analyses. As little as 25% of the normal fibroblasts' enzyme levels were sufficient for this correction. The half-disappearance time of the internalized enzyme was 3-4 days. Prolonged incubation of corrected cells resulted in a gradual reaccumulation of mucopolysaccharides. SN - 0002-9297 UR - https://www.unboundmedicine.com/medline/citation/6789675/The_correction_of_Hunter_fibroblasts_by_exogenous_iduronate_sulfate_sulfatase:_biochemical_and_ultrastructural_studies_ L2 - https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/6789675/ DB - PRIME DP - Unbound Medicine ER -