Huizing Marjan, Helip-Wooley Amanda, Westbroek Wendy, Gunay-Aygun Meral, Gahl William A
Cell Biology of Metabolic Disorders Unit, National Institutes of Health, Bethesda, Maryland 20892, USA.
Annu Rev Genomics Hum Genet. 2008;9:359-86. doi: 10.1146/annurev.genom.9.081307.164303.
Lysosome-related organelles (LROs) are a heterogeneous group of vesicles that share various features with lysosomes, but are distinct in function, morphology, and composition. The biogenesis of LROs employs a common machinery, and genetic defects in this machinery can affect all LROs or only an individual LRO, resulting in a variety of clinical features. In this review, we discuss the main components of LRO biogenesis. We also summarize the function, composition, and resident cell types of the major LROs. Finally, we describe the clinical characteristics of the major human LRO disorders.
溶酶体相关细胞器(LROs)是一类异质性的囊泡,它们与溶酶体具有多种共同特征,但在功能、形态和组成上有所不同。LROs的生物发生利用了一套共同的机制,该机制中的基因缺陷可影响所有LROs或仅影响单个LRO,从而导致多种临床特征。在本综述中,我们讨论了LRO生物发生的主要成分。我们还总结了主要LROs的功能、组成和驻留细胞类型。最后,我们描述了主要人类LRO疾病的临床特征。