{"doi":"10.3410/f.735464281.793574979","title":"Faculty Opinions recommendation of The road to lysosome-related organelles: Insights from Hermansky-Pudlak syndrome and other rare diseases.","abstract":"Lysosome-related organelles comprise a diverse group of cell type-specific, membrane-bound subcellular organelles that derive at least in part from the endolysosomal system but that have unique contents, morphology, and functions to support specific physiological roles. They include melanosomes that provide pigment to our eyes and skin, alpha and dense granules in platelets and lytic granules in cytotoxic T cells and natural killer cells that release effectors to regulate hemostasis and immunity, and distinct classes of lamellar bodies in lung epithelial cells and keratinocytes that support lung plasticity and skin lubrication. The formation, maturation and/or secretion of subsets of lysosome-related organelles are dysfunctional or entirely absent in a number of hereditary syndromic disorders, including in particular the Hermansky-Pudlak syndromes. This review provides a comprehensive overview of lysosome-related organelles in humans and model organisms and presents our current understanding of how the products of genes that are defective in heritable diseases impact their formation, motility and ultimate secretion.","journal":"Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature","year":2020,"id":134139,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9564,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":427417,"name":"Norihiko Ohbayashi","orcid":null,"position":1,"is_corresponding":false},{"id":501065,"name":"Mitsunori Fukuda","orcid":"0000-0002-8620-5853","position":0,"is_corresponding":true}],"reference_count":356,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-18T23:16:24.540681Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}