Marks M S, Seabra M C
Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA 19104-6082, USA.
Nat Rev Mol Cell Biol. 2001 Oct;2(10):738-48. doi: 10.1038/35096009.
Melanosomes are morphologically and functionally unique organelles within which melanin pigments are synthesized and stored. Melanosomes share some characteristics with lysosomes, but can be distinguished from them in many ways. The biogenesis and intracellular movement of melanosomes and related organelles are disrupted in several genetic disorders in mice and humans. The recent characterization of genes defective in these diseases has reinvigorated interest in the melanosome as a model system for understanding the molecular mechanisms that underlie intracellular membrane dynamics.
黑素小体是形态和功能独特的细胞器,黑色素在此合成并储存。黑素小体与溶酶体有一些共同特征,但在许多方面又可与之区分。在小鼠和人类的几种遗传疾病中,黑素小体及相关细胞器的生物发生和细胞内移动受到破坏。最近对这些疾病中缺陷基因的表征,重新激发了人们对黑素小体作为理解细胞内膜动力学潜在分子机制的模型系统的兴趣。