黏脂贮积症IV型患者来源的培养皮肤成纤维细胞中的黏多糖积累。
Mucopolysaccharide accumulation in cultured skin fibroblasts derived from patients with mucolipidosis IV.
作者信息
Bach G, Ziegler M, Kohn G, Cohen M M
出版信息
Am J Hum Genet. 1977 Nov;29(6):610-8.
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.
摘要
与正常对照组相比，在IV型粘脂贮积症（ML IV）成纤维细胞提取物中发现总硫酸化粘多糖（MPS）浓度增加了三倍，透明质酸（HA）浓度增加了10倍。这种积累改变了MPS的分布：在ML IV中，MPS:HA占总MPS的70%，而在对照细胞中仅占30%。在ML IV成纤维细胞中观察到细胞内硫酸化MPS几乎呈线性积累。“脉冲追踪”实验表明，HA和硫酸化MPS在ML IV细胞中都能长时间留存；在对照细胞中，它们会迅速作为低分子量、可透析的片段被清除。这些数据表明，ML IV成纤维细胞中MPS的积累是分解代谢受阻的结果，可能与溶酶体有关。
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