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    Am J Hum Genet. 1977 Nov;29(6):610-8.

    Mucopolysaccharide accumulation in cultured skin fibroblasts derived from patients with mucolipidosis IV.

    Bach G, Ziegler M, Kohn G, Cohen MM.

    Abstract

    Increased concentrations of total sulfated mucopolysaccharides (MPS), threefold, and hyaluronic acid (HA), 10-fold, were found in ML IV fibroblast extracts when compared to normal controls. Such accumulations altered the distribution of MPS:HA comprised 70% of total MPS in ML IV but only 30% in control cells. Intracellular sulfated MPS was observed accumulating almost linearly in ML IV fibroblasts. "Pulse-chase" experiments indicate that both HA and the sulfated MPS remain in the ML IV cells for long periods of time; in control cells, they are rapidly removed as low molecular weight, dialyzable fragments. These data suggest that the MPS accumulation in ML IV fibroblasts, is the consequence of a catabolic block, probably involving the lysosome.

    PMID:
    145180
    [PubMed - indexed for MEDLINE]
    PMCID:
    PMC1685507
    Free PMC Article

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