{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:21:32Z","timestamp":1760145692025,"version":"build-2065373602"},"reference-count":66,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T00:00:00Z","timestamp":1723593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008061","name":"Fundaci\u00f3n Mutua Madrile\u00f1a","doi-asserted-by":"publisher","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}],"id":[{"id":"10.13039\/100008061","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"name":"European Union","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}]},{"name":"Ministerio de Ciencia e Innovaci\u00f3n","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}]},{"name":"Personalized and Precision Medicine","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}]},{"name":"Instituto de Salud Carlos III","award":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"],"award-info":[{"award-number":["AP183072023","PI23\/00175","PMP22\/00092","CD21\/00059"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Sanfilippo syndrome, or mucopolysaccharidosis type III (MPS III), is a rare lysosomal disease caused by congenital enzymatic deficiencies in heparan sulfate (HS) degradation, leading to organ dysfunction. The most severe hallmark of MPS III comprises neurological alterations, although gastrointestinal symptoms (GISs) have also been shown to be relevant in many patients. Here, we explored the contribution of the gut microbiota to MPS III GISs. We analyzed the composition and functionality of the gut microbiota in two MPS III siblings with the same mutation (c.544C &gt; T, c.1080delC, in the SGSH gene) and the same diet, but with differences in their GISs, including recurrent diarrhea in one of them. Using 16S sequencing, we observed that the MPS III patients exhibited decreased alpha diversity and a lower abundance of Lachnospiraceae and Bifidobacteriaceae accompanied by a higher abundance of the Ruminococcaceae and Rikenellaceae families than the healthy control subjects. Comparing siblings, we found an increased abundance of Bacteroidaceae and a lower abundance of Ruminococcaceae and Akkermansiaceae in the GIS-free patient. This patient also had a higher relative abundance of Sus genes (SusA, SusB, SusE, and SusG) involved in glycosaminoglycan metabolism. We found higher HS levels in the stool of the two MPS III patients than in healthy volunteers, particularly in the patient with GISs. Functionally, whole fecal metabolites from the patient with GISs induced oxidative stress in vitro in healthy monocytes. Finally, the Bacteroides\u00a0thetaiotaomicron strain isolated from MPS III stool samples exhibited HS degradation ability. Overall, our results reveal different microbiota compositions and functionalities in MPS III siblings, who exhibited differential gastrointestinal symptomatology. Our study may serve as a gateway to explore the impact of the gut microbiota and its potential to enhance the quality of life in Sanfilippo syndrome patients.<\/jats:p>","DOI":"10.3390\/ijms25168856","type":"journal-article","created":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T10:28:41Z","timestamp":1723631321000},"page":"8856","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Role of the Gut Microbiota in Sanfilippo Syndrome\u2019s Physiopathology: An Approach in Two Affected Siblings"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8845-1137","authenticated-orcid":false,"given":"Raquel","family":"Barbero-Herranz","sequence":"first","affiliation":[{"name":"Microbiology Department, Hospital Universitario Ram\u00f3n y Cajal and Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4683-2043","authenticated-orcid":false,"given":"Mar\u00eda","family":"Garriga-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Endocrinology and Nutrition Service, Hospital Universitario Ram\u00f3n y Cajal, 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3220-1117","authenticated-orcid":false,"given":"Ana","family":"Moreno-Blanco","sequence":"additional","affiliation":[{"name":"Microbiology Department, Hospital Universitario Ram\u00f3n y Cajal and Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"},{"name":"CIBER de Enfermedades Infecciosas, Instituto de Salud Carlos III, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esther","family":"Palacios","sequence":"additional","affiliation":[{"name":"Microbiology Department, Hospital Universitario Ram\u00f3n y Cajal and Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1785-4988","authenticated-orcid":false,"given":"Pedro","family":"Ruiz-Sala","sequence":"additional","affiliation":[{"name":"Centro de Diagn\u00f3stico de Enfermedades Moleculares (CEDEM), Autonomous University of Madrid (UAM), IdiPaz, 28049 Madrid, Spain"},{"name":"CIBER de Enfermedades Raras, Instituto de Salud Carlos III, 28049 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saioa","family":"Vicente-Santamar\u00eda","sequence":"additional","affiliation":[{"name":"Endocrinology and Nutrition Service, Hospital Universitario Ram\u00f3n y Cajal, 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0340-4580","authenticated-orcid":false,"given":"Sinziana","family":"Stanescu","sequence":"additional","affiliation":[{"name":"Unidad de Enfermedades Metab\u00f3licas Hospital, CSUR, MetabERN, Pediatric Department, Hospital Universitario Ram\u00f3n y Cajal, Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amaya","family":"Belanger-Quintana","sequence":"additional","affiliation":[{"name":"Unidad de Enfermedades Metab\u00f3licas Hospital, CSUR, MetabERN, Pediatric Department, Hospital Universitario Ram\u00f3n y Cajal, Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9347-2386","authenticated-orcid":false,"given":"Guillem","family":"Pintos-Morell","sequence":"additional","affiliation":[{"name":"Vall d\u2019Hebron Institut de Recerca (VHIR), Unidad de Enfermedades Raras, Hospital Vall d\u2019Hebron Barcelona Hospital Campus, Comit\u00e9 M\u00e9dico Consultivo MPS-Lisosomales, 08035 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatriz","family":"Arconada","sequence":"additional","affiliation":[{"name":"Federaci\u00f3n Espa\u00f1ola de Enfermedades Raras (FEDER), 28009 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1147-7923","authenticated-orcid":false,"given":"Rosa","family":"del Campo","sequence":"additional","affiliation":[{"name":"Microbiology Department, Hospital Universitario Ram\u00f3n y Cajal and Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"},{"name":"CIBER de Enfermedades Infecciosas, Instituto de Salud Carlos III, 28029 Madrid, Spain"},{"name":"Faculty of Health Sciences, Alfonso X El Sabio University, Villanueva de la Ca\u00f1ada, 28691 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2774-1821","authenticated-orcid":false,"given":"Jos\u00e9","family":"Avenda\u00f1o-Ortiz","sequence":"additional","affiliation":[{"name":"Microbiology Department, Hospital Universitario Ram\u00f3n y Cajal and Instituto Ram\u00f3n y Cajal de Investigaci\u00f3n Sanitaria (IRYCIS), 28034 Madrid, Spain"},{"name":"CIBER de Enfermedades Infecciosas, Instituto de Salud Carlos III, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,8,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1111\/ped.12636","article-title":"Sanfilippo Syndrome: Overall Review","volume":"57","author":"Andrade","year":"2015","journal-title":"Pediatr. 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