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首页> 外文期刊>Pigment cell research >Membranous complexes characteristic of melanocytes derived from patients with Hermansky-Pudlak syndrome type 1 are macroautophagosomal entities of the lysosomal compartment
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Membranous complexes characteristic of melanocytes derived from patients with Hermansky-Pudlak syndrome type 1 are macroautophagosomal entities of the lysosomal compartment

机译：源自1型Hermansky-Pudlak综合征患者的黑色素细胞的膜复合物是溶酶体区室的巨自噬体

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摘要

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder resulting from mutations in a family of genes required for efficient transport of lysosomal-related proteins from the trans-Golgi network to a target organelle. To date, there are several genetically distinct forms of HPS. Many forms of HPS exhibit aberrant trafficking of melanosome-targeted proteins resulting in incomplete melanosome biogenesis responsible for oculocutaneous albinism observed in patients. In HPS-1, melanosome-targeted proteins are localized to characteristic membranous complexes, which have morphologic similarities to macroautophagosomes. In this report, we evaluated the hypothesis that HPS-1-specific membranous complexes comprise a component of the lysosomal compartment of melanocytes. Using indirect immunofluorescence, an increase in co-localization of misrouted tyrosinase with cathepsin-L, a lysosomal cysteine protease, occurred in HPS-1 melanocytes. In addition, ribophorin II, an integral endoplasmic reticulum protein that is also a component of macroautophagosomes, and LC3, a specific marker of macrophagosomes, demonstrated localization to membranous complexes in HPS-1 melanocytes. At the electron microscopic level, the membranous complexes exhibited acid phosphatase activity and localization of exogenously supplied horseradish peroxidase (HRP)-conjugated gold particles, indicating incorporation of lysosomal and endosomal components to membranous complexes, respectively. These results confirm that membranous complexes of HPS-1 melanocytes are macroautophagosomal representatives of the lysosomal compartment.
机译：Hermansky-Pudlak综合征（HPS）是一种常染色体隐性遗传疾病，是由于溶酶体相关蛋白从反式高尔基体网络有效转运至靶细胞器所需的基因家族突变引起的。迄今为止，HPS有几种遗传上不同的形式。许多形式的HPS表现出以黑素体为靶标的蛋白的异常运输，导致黑素体生物发生不完全，从而导致患者眼白化病。在HPS-1中，针对黑素体的蛋白质定位于特征性膜复合物，该复合物的形态与大型自噬体相似。在本报告中，我们评估了以下假设：HPS-1特异性膜状复合物包含黑素细胞溶酶体区室的成分。使用间接免疫荧光，在HPS-1黑色素细胞中，错误途径的酪氨酸酶与组织蛋白酶-L（一种溶酶体半胱氨酸蛋白酶）的共定位增加。此外，核糖体蛋白II（一种内质网蛋白，也是巨噬细胞的一个组成部分）和LC3（巨噬细胞的一种特异性标记）在HPS-1黑色素细胞中定位于膜状复合物。在电子显微镜下，膜状复合物表现出酸性磷酸酶活性和外源供应的辣根过氧化物酶（HRP）-共轭的金颗粒的定位，表明溶酶体和内体组分分别结合到膜状复合物中。这些结果证实，HPS-1黑色素细胞的膜复合物是溶酶体区室的大自噬体代表。

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