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This study investigated the effects of defective LYST on neurodegenerative features by the morphological analysis of organelles in dopaminergic neurons differentiated from induced pluripotent stem cells (iPSCs) derived from CHS patients.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>iPSCs derived from CHS patients were analyzed by immunostaining with antibodies against microtubule\u2010associated protein 2 and tyrosine hydroxylase, periodic acid\u2010Schiff (PAS) staining, electron microscopy, and staining with a fluorescent probe to monitor autophagy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>iPSC\u2010derived neurons contained PAS\u2010positive giant granules and lipofuscin\u2010like granules. Electron microscopy revealed enlarged lysosomes with electron\u2010dense granules and filament\u2010like structures. The number and brightness of autophagosomes and autolysosomes were markedly increased in CHS iPSC\u2010derived neurons under basal culture conditions. CHS iPSC\u2010derived neuronal mitochondria were enlarged, polymorphic, and hypertrophic.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>CHS iPSC\u2010derived neurons contained abnormal organelles, including lysosomes, autophagosomes, and mitochondria, which may be related to the neurodegenerative features of CHS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ped.70326","type":"journal-article","created":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T05:31:46Z","timestamp":1771392706000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Analysis of intracellular organelles in neurons differentiated from\n                    <scp>iPSCs<\/scp>\n                    of Ch\u00e9diak\u2010Higashi syndrome patients"],"prefix":"10.1111","volume":"68","author":[{"given":"Yasuko","family":"Nakamura","sequence":"first","affiliation":[{"name":"Department of Pediatrics National Defense Medical College  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masataka","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Developmental Anatomy and Regenerative Biology National Defense Medical College  Saitama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Senoo","sequence":"additional","affiliation":[{"name":"The Department of Pathology BML, Inc.  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