Overexpression of wild-type and mutant mucolipin proteins in mammalian cells: effects on the late endocytic compartment organization
Overexpression of wild-type and mutant mucolipin proteins in mammalian cells: effects on the late endocytic compartment organization
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DOI：
10.1016/j.febslet.2004.04.080
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发表时间：
2004-06-04
期刊：
影响因子：
3.5
通讯作者：
Borsani, G
中科院分区：
文献类型：
--
作者：
Manzoni, M;Monti, E;Borsani, G
粘脂蛋白-1是一种由MCOLN 1基因编码的65-kDa膜蛋白，该基因在IV型粘脂沉积症（MLIV）患者中发生突变，MLIV是一种罕见的神经退行性溶酶体储存疾病。我们通过在HeLa细胞中表达Myc标记的蛋白质，研究了野生型和三种不同突变形式（T232 P，F408 del和F465 L）的粘脂的亚细胞定位。过表达的野生型粘脂共定位于晚期内吞结构，并诱导这些隔室的异常分布。F408 del和F465 L MLIV突变蛋白显示与野生型蛋白相似的分布，而T232 P保留在内质网中。在突变体中，只有F408 del诱导晚期内吞隔室的再分配。这些结果表明，过度表达的粘脂阳离子通道影响晚期内吞室的动态平衡。(C)2004年欧洲生物化学学会联合会。Elsevier B. V.出版，保留所有权利。
Mucolipin-1 is a 65-kDa membrane protein encoded by the MCOLN1 gene, which is mutated in patients with mucolipidosis type IV (MLIV), a rare neurodegenerative lysosomal storage disorder. We studied the subcellular localization of wild-type and three different mutant forms (T232P, F408del and F465L) of mucolipin by expressing Myc-tagged proteins in HeLa cells. The overexpressed wild-type mucolipin colocalizes to late endocytic structures and induces an aberrant distribution of these compartments. F408del and F465L MLIV mutant proteins show a distribution similar to the wild-type protein, whereas T232P is retained in the endoplasmic reticulum. Among the mutants, only F408del induces a redistribution of the late endocyctic compartment. These findings suggest that the overexpression of the mucolipin cation channel influences the dynamic equilibrium of late endocytic compartments. (C) 2004 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.