{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:16:54Z","timestamp":1787015814799,"version":"build-2736575974"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T00:00:00Z","timestamp":1770854400000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neurogastroenterology Motil"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Chronic intestinal pseudo\u2010obstruction (CIPO) occurs in up to 40% of patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke\u2010like episodes (MELAS). While enteric nervous system abnormalities have been documented, 15%\u201338% of CIPO cases show normal histology, suggesting alternative pathogenic mechanisms. We aimed to investigate smooth muscle pathology in MELAS\u2010associated CIPO.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Comprehensive pathological examination including light and electron microscopy was performed on autopsy material from a 52\u2010year\u2010old male with MELAS (m.3243A &gt; G mutation) and recurrent intestinal obstruction symptoms. Gastrointestinal tissue from the entire digestive tract was analyzed and compared with age\u2010matched control tissue.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Key Results<\/jats:title>\n                    <jats:p>Histological examination revealed widespread vacuolization and pallor of gastrointestinal smooth muscle throughout the digestive tract (esophagus, stomach, duodenum, and colon). Electron microscopy demonstrated abundant abnormal mitochondria in smooth muscle cells and, notably, marked loss of myofilaments in the colonic muscle. The ultrastructural preservation was limited by postmortem changes (autolysis, occurring approximately 4\u2009h after death) and the re\u2010embedding technique from formalin\u2010fixed paraffin\u2010embedded tissue; however, the striking difference was evident compared to age\u2010matched controls. Similar abnormalities were observed in the Auerbach plexus.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions and Inferences<\/jats:title>\n                    <jats:p>This represents the first report of myofilament loss in MELAS\u2010associated CIPO, suggesting that mitochondrial dysfunction may directly impair smooth muscle contractile apparatus beyond previously described neuronal abnormalities. These findings provide novel insights into CIPO pathogenesis and may inform therapeutic strategies emphasizing early enteral nutrition interventions that bypass affected gastrointestinal segments.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/nmo.70226","type":"journal-article","created":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T04:39:51Z","timestamp":1770957591000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Loss of Myofilaments in Gastrointestinal Smooth Muscle: A Novel Pathological Finding in\n                    <scp>MELAS<\/scp>\n                    \u2010Associated Chronic Intestinal Pseudo\u2010Obstruction"],"prefix":"10.1111","volume":"38","author":[{"given":"Satoaki","family":"Nagao","sequence":"first","affiliation":[{"name":"Department of Pathology, School of Medicine Fukuoka University  Fukuoka Japan"},{"name":"Department of Diabetes, Endocrinology and Metabolism Kitasato University School of Medicine  Sagamihara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikiko","family":"Aoki","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine Fukuoka University  Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4064-3102","authenticated-orcid":false,"given":"Shuichi","family":"Yatsuga","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine Fukuoka University  Fukuoka Japan"},{"name":"Department of Medical Genetics Hakodate Goryoukaku Hospital  Hakodate Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sho","family":"Takeshita","sequence":"additional","affiliation":[{"name":"Department of Neurology Fukuoka University Chikushi Hospital  Chikushino Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshio","family":"Tsuboi","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medicine Fukuoka University  Fukuoka Japan"},{"name":"Tsutsumi Clinic Fukuoka Parkinson's Disease Center  Fukuoka Japan"},{"name":"Department of Neurology, Long\u2010Term Observation Research Juntendo University  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriko","family":"Uesugi","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine Fukuoka University  Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,12]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2982.2007.00902.x"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.06.004"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e3282ef58cd"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.41.5.675"},{"key":"e_1_2_12_6_1","first-page":"775","article-title":"Chronic Intestinal Pseudoobstruction due to Mitochondrial Myopathy: Possible Relationship of Nitric Oxide With Intestinal Motility","volume":"35","author":"Mori M.","year":"2000","journal-title":"Journal of Gastroenterology"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1159\/000337528"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v18.i33.4557"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-201X.1998.00441.x"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00425.2001"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00253.2008"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/bs.apha.2017.06.001"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7793.2000.00865.x"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.12998\/wjcc.v11.i15.3369"},{"key":"e_1_2_12_15_1","first-page":"111","article-title":"Superior Mesenteric Artery Syndrome: Clinical and Radiological Considerations","volume":"107","author":"Bartel T. 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