Delevoye Cédric, Giordano Francesca, van Niel Guillaume, Raposo Graça
Institut Curie, Centre de recherche, CNRS UMR144, 26, rue d'Ulm, 75248 Paris, France.
Med Sci (Paris). 2011 Feb;27(2):153-62. doi: 10.1051/medsci/2011272153. Epub 2011 Mar 8.
Melanosomes are lysosome-related organelles in retinal pigment epithelial cells and epidermal melanocytes in which melanin pigments are synthesized and stored. Melanosomes are generated by multistep processes in which an immature unpigmented organelle forms and then subsequently matures. Such maturation requires inter-organellar transport of protein cargos required for pigment synthesis but also recruitment of effector proteins necessary for the correct transport of melanosomes to the cell periphery. Several studies have started to unravel the main pathways and mechanisms exploited by melanosomal proteins involved in melanosome structure and melanin synthesis. A major unexpected finding seen early in melanosome biogenesis showed the similarities between the fibrillar sheets of premelanosomes and amyloid fibrils. Late steps of melanosome formation are dependent on pathways regulated by proteins encoded by genes mutated in genetic diseases such as the Hermansky-Pudlak Syndrom (HPS) and different types of albinism. Altogether the findings from the past recent years have started to unravel how specialized cells integrate unique and ubiquitous molecular mechanisms in subverting the endosomal system to generate cell-type specific structures and their associated functions. Further dissection of the melanosomal system will likely shed light not only on the biogenesis of lysosome-related organelles but also on general aspects of vesicular transport in the endosomal system.
黑素小体是视网膜色素上皮细胞和表皮黑素细胞中与溶酶体相关的细胞器,黑色素在其中合成并储存。黑素小体通过多步骤过程生成,即先形成未成熟的无色素细胞器,随后成熟。这种成熟不仅需要色素合成所需蛋白质货物的细胞器间运输,还需要募集将黑素小体正确运输到细胞周边所需的效应蛋白。几项研究已开始揭示参与黑素小体结构和黑色素合成的黑素小体蛋白所利用的主要途径和机制。在黑素小体生物发生早期发现的一个主要意外结果是前黑素小体的纤维状片层与淀粉样纤维之间存在相似性。黑素小体形成的后期步骤依赖于由遗传性疾病(如赫尔曼斯基-普德拉克综合征(HPS)和不同类型白化病)中突变基因编码的蛋白质所调控的途径。近年来的研究结果总体上已开始揭示特化细胞如何整合独特且普遍存在的分子机制来颠覆内体系统,以产生细胞类型特异性结构及其相关功能。对黑素小体系统的进一步剖析可能不仅会阐明与溶酶体相关细胞器的生物发生,还会揭示内体系统中囊泡运输的一般方面。