{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T07:14:31Z","timestamp":1760426071466,"version":"build-2065373602"},"reference-count":34,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,9,16]],"date-time":"2022-09-16T00:00:00Z","timestamp":1663286400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico (CNPq)","award":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"],"award-info":[{"award-number":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"]}]},{"DOI":"10.13039\/501100004263","name":"Funda\u00e7\u00e3o de Amparo \u00e0 Pesquisa do Estado do Rio Grande do Sul (FAPERGS)","doi-asserted-by":"publisher","award":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"],"award-info":[{"award-number":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"]}],"id":[{"id":"10.13039\/501100004263","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003810","name":"Fundo de Incentivo \u00e0 Pesquisa e Eventos do Hospital de Cl\u00ednicas de Porto Alegre","doi-asserted-by":"publisher","award":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"],"award-info":[{"award-number":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"]}],"id":[{"id":"10.13039\/501100003810","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior\u2014Brasil (CAPES)","doi-asserted-by":"publisher","award":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"],"award-info":[{"award-number":["EDITAL PRONEX FAPERGS\/CNPq 12\/2014","16\/2551-0000492-7","20150218","20190457","001"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Metabolites"],"abstract":"<jats:p>Recently, patients with glycogen storage disease (GSD) have been described as having gut dysbiosis, lower fecal pH, and an imbalance in SCFAs due to an increase in acetate and propionate levels. Here, we report the fecal measurement of bacterial-related metabolites formic, acetic, lactic, propionic, and succinic acid, a key metabolite of both host and microbiota, on a previously described cohort of 24 patients (GSD Ia = 15, GSD Ib = 5, 1 GSD III = 1 and GSD IX = 3) and 16 healthy controls, with similar sex and age, using the high-performance liquid chromatography technique. The succinic acid levels were higher in the GSD patients than in the controls (patients = 38.02; controls = 27.53; p = 0.045), without differences between the groups for other metabolites. Fecal pH present inverse correlation with lactic acid (R = \u22120.54; p = 0.0085), while OTUs were inversely correlated with both lactic (R = \u22120.46; p = 0.026) and formic (R = \u22120.54; p = 0.026) acids. Using two distinct metrics of diversity, borderline significance was obtained for propionic acid, affecting the microbial structure on Euclidean basis in 8% (r2 = 0.081; p = 0.079), and for lactic acid, affecting 6% of microbial structure using Bray\u2013Curtis distance (r2 = 0.065; p = 0.060). No correlation was found between SCFAs and total carbohydrate consumption among the participants or uncooked cornstarch consumption among the patients.<\/jats:p>","DOI":"10.3390\/metabo12090873","type":"journal-article","created":{"date-parts":[[2022,9,19]],"date-time":"2022-09-19T02:06:50Z","timestamp":1663553210000},"page":"873","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Are the Bacteria and Their Metabolites Contributing for Gut Inflammation on GSD-Ia Patients?"],"prefix":"10.3390","volume":"12","author":[{"given":"Karina","family":"Colonetti","sequence":"first","affiliation":[{"name":"Laboratory of Basic Research and Advanced Investigations in Neurosciences (BRAIN), Porto Alegre 90035-903, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evelise Leis","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Prote\u00f4mica Aplicada (LPA), Universidade Federal do Pampa, S\u00e3o Gabriel 97300-000, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Darlene Lopes","family":"Rangel","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Prote\u00f4mica Aplicada (LPA), Universidade Federal do Pampa, S\u00e3o Gabriel 97300-000, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8513-0335","authenticated-orcid":false,"given":"Paulo Marcos","family":"Pinto","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Prote\u00f4mica Aplicada (LPA), Universidade Federal do Pampa, S\u00e3o Gabriel 97300-000, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1450-8828","authenticated-orcid":false,"given":"Luiz Fernando Wurdig","family":"Roesch","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Cell Science, Institute of Food and Agricultural Sciences, University of Florida, Gainesville 32611, FL, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0333-0350","authenticated-orcid":false,"given":"Franciele Cabral","family":"Pinheiro","sequence":"additional","affiliation":[{"name":"Laboratory of Basic Research and Advanced Investigations in Neurosciences (BRAIN), Porto Alegre 90035-903, RS, Brazil"},{"name":"Laborat\u00f3rio de Biologia, Universidade Federal do Pampa, Itaqui 97650-000, RS, Brazil"},{"name":"Post-Graduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre 91501-970, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ida Vanessa Doederlein","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Laboratory of Basic Research and Advanced Investigations in Neurosciences (BRAIN), Porto Alegre 90035-903, RS, Brazil"},{"name":"Post-Graduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre 91501-970, RS, Brazil"},{"name":"Medical Genetics Service, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre 90035-903, RS, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.pcl.2017.11.005","article-title":"Inborn Errors of Metabolism with Hypoglycemia: Glycogen Storage Diseases and Inherited Disorders of Gluconeogenesis","volume":"65","author":"Weinstein","year":"2018","journal-title":"Pediatr. 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