Journal ArticleDOI
Recognition and receptor-mediated uptake of a lysosomal enzyme, α-l-iduronidase, by cultured human fibroblasts
TLDR
Preliminary experiments suggest that even the "low uptake" form of α-l-iduronidase may be taken up by receptor binding, although with much lower affinity.About:
This article is published in Cell.The article was published on 1977-11-01. It has received 331 citations till now. The article focuses on the topics: Mannose & Alkaline phosphatase.read more
Citations
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Journal ArticleDOI
Endocytosis and the recycling of plasma membrane.
TL;DR: This article centers on the properties and dynamics of the endocytic vacuole membrane and stresses observations on cultured mouse macrophages with which the authors are most familier.
Journal ArticleDOI
Intracellular protein topogenesis.
TL;DR: A cell contains millions of protein molecules, which are continually being synthesized and degraded, and at homeostasis, a given species of protein is represented by a characteristic number of molecules that is kept constant within a narrow range.
Journal ArticleDOI
Mechanisms for the incorporation of proteins in membranes and organelles.
TL;DR: Mechanisms for the transfer of newly synthesized polypeptides to their sites of function in different subcellular membranes and organelles are considered, and models in which specific features of the polypePTides serve as signals to direct them along selected sub cellular pathways to their final destination are discussed.
References
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Book ChapterDOI
The role of surface carbohydrates in the hepatic recognition and transport of circulating glycoproteins.
Gilbert Ashwell,Anatol G. Morell +1 more
Journal ArticleDOI
Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts
TL;DR: Evidence is presented that alkaline phosphatase treatment of human platelet beta-glucuronidase abolished its "high-uptake" activity, without diminishing its catalytic activity, and converted some forms of the heterogeneous enzyme to less acidic forms.
Journal ArticleDOI
Familial hypercholesterolemia: Defective binding of lipoproteins to cultured fibroblasts associated with impaired regulation of 3-hydroxy-3-methylglutaryl coenzyme a reductase activity
TL;DR: The demonstration of a defect in binding of low-density lipoproteins to cells from subjects with the homozygous form of familial hypercholesterolemia appears to explain the previously reported failure of lipoproteinins to suppress the synthesis of this enzyme and hence may account for the overproduction of cholesterol that occurs in these cultured cells.
Journal ArticleDOI
A hypothesis for I-cell disease: Defective hydrolases that do not enter lysosomes
TL;DR: It is suggested that the packaging of lysosomal enzymes requires their secretion followed by specific recognition and uptake, and the I-cell mutation would interfere with this process by altering the recognition site on the hydrolases.