Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics.
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ABSTRACT:
SUBMITTER: Huizing M
PROVIDER: S-EPMC2755194 | biostudies-literature | 2008
REPOSITORIES: biostudies-literature
Publications
Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics.
Huizing Marjan M Helip-Wooley Amanda A Westbroek Wendy W Gunay-Aygun Meral M Gahl William A WA
Annual review of genomics and human genetics 20080101
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 m ...[more]
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