Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients

A life-long dietary intervention can affect the substrates’ availability for gut fermentation in metabolic diseases such as the glycogen-storage diseases (GSD). Besides drug consumption, the main treatment of types GSD-Ia and Ib to prevent metabolic complications is a specific diet with definite nut...

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Main Authors: Camilla Ceccarani, Giulia Bassanini, Chiara Montanari, Maria Cristina Casiraghi, Emerenziana Ottaviano, Giulia Morace, Giacomo Biasucci, Sabrina Paci, Elisa Borghi, Elvira Verduci
Format: Article
Language:English
Published: MDPI AG 2020-03-01
Series:Metabolites
Subjects:
Online Access:https://www.mdpi.com/2218-1989/10/4/133
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author Camilla Ceccarani
Giulia Bassanini
Chiara Montanari
Maria Cristina Casiraghi
Emerenziana Ottaviano
Giulia Morace
Giacomo Biasucci
Sabrina Paci
Elisa Borghi
Elvira Verduci
author_facet Camilla Ceccarani
Giulia Bassanini
Chiara Montanari
Maria Cristina Casiraghi
Emerenziana Ottaviano
Giulia Morace
Giacomo Biasucci
Sabrina Paci
Elisa Borghi
Elvira Verduci
author_sort Camilla Ceccarani
collection DOAJ
description A life-long dietary intervention can affect the substrates’ availability for gut fermentation in metabolic diseases such as the glycogen-storage diseases (GSD). Besides drug consumption, the main treatment of types GSD-Ia and Ib to prevent metabolic complications is a specific diet with definite nutrient intakes. In order to evaluate how deeply this dietary treatment affects gut bacteria, we compared the gut microbiota of nine GSD-I subjects and 12 healthy controls (HC) through 16S rRNA gene sequencing; we assessed their dietary intake and nutrients, their microbial short chain fatty acids (SCFAs) via gas chromatography and their hematic values. Both alpha-diversity and phylogenetic analysis revealed a significant biodiversity reduction in the GSD group compared to the HC group, and highlighted profound differences of their gut microbiota. GSD subjects were characterized by an increase in the relative abundance of <i>Enterobacteriaceae</i> and <i>Veillonellaceae</i> families, while the beneficial genera <i>Faecalibacterium</i> and <i>Oscillospira</i> were significantly reduced. SCFA quantification revealed a significant increase of fecal acetate and propionate in GSD subjects, but with a beneficial role probably reduced due to unbalanced bacterial interactions; nutritional values correlated to bacterial genera were significantly different between experimental groups, with nearly opposite cohort trends.
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spelling doaj.art-ea4f971ac0c74bfe9f9505806d7efb2b2023-11-19T20:11:16ZengMDPI AGMetabolites2218-19892020-03-0110413310.3390/metabo10040133Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 PatientsCamilla Ceccarani0Giulia Bassanini1Chiara Montanari2Maria Cristina Casiraghi3Emerenziana Ottaviano4Giulia Morace5Giacomo Biasucci6Sabrina Paci7Elisa Borghi8Elvira Verduci9Department of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyDepartment of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyDepartment of Pediatrics, San Paolo Hospital, 20142 Milan, ItalyDepartment of Food, Environmental and Nutritional Sciences, Università degli Studi di Milano, 20133 Milan, ItalyDepartment of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyDepartment of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyDepartment of Pediatrics and Neonatology, Guglielmo da Saliceto Hospital, 29121 Piacenza, ItalyDepartment of Pediatrics, San Paolo Hospital, 20142 Milan, ItalyDepartment of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyDepartment of Health Sciences, Università degli Studi di Milano, 20142 Milan, ItalyA life-long dietary intervention can affect the substrates’ availability for gut fermentation in metabolic diseases such as the glycogen-storage diseases (GSD). Besides drug consumption, the main treatment of types GSD-Ia and Ib to prevent metabolic complications is a specific diet with definite nutrient intakes. In order to evaluate how deeply this dietary treatment affects gut bacteria, we compared the gut microbiota of nine GSD-I subjects and 12 healthy controls (HC) through 16S rRNA gene sequencing; we assessed their dietary intake and nutrients, their microbial short chain fatty acids (SCFAs) via gas chromatography and their hematic values. Both alpha-diversity and phylogenetic analysis revealed a significant biodiversity reduction in the GSD group compared to the HC group, and highlighted profound differences of their gut microbiota. GSD subjects were characterized by an increase in the relative abundance of <i>Enterobacteriaceae</i> and <i>Veillonellaceae</i> families, while the beneficial genera <i>Faecalibacterium</i> and <i>Oscillospira</i> were significantly reduced. SCFA quantification revealed a significant increase of fecal acetate and propionate in GSD subjects, but with a beneficial role probably reduced due to unbalanced bacterial interactions; nutritional values correlated to bacterial genera were significantly different between experimental groups, with nearly opposite cohort trends.https://www.mdpi.com/2218-1989/10/4/133glycogen storage diseaseGSDdietgut microbiotashort-chain fatty acidsinflammation
spellingShingle Camilla Ceccarani
Giulia Bassanini
Chiara Montanari
Maria Cristina Casiraghi
Emerenziana Ottaviano
Giulia Morace
Giacomo Biasucci
Sabrina Paci
Elisa Borghi
Elvira Verduci
Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
Metabolites
glycogen storage disease
GSD
diet
gut microbiota
short-chain fatty acids
inflammation
title Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
title_full Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
title_fullStr Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
title_full_unstemmed Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
title_short Proteobacteria Overgrowth and Butyrate-Producing Taxa Depletion in the Gut Microbiota of Glycogen Storage Disease Type 1 Patients
title_sort proteobacteria overgrowth and butyrate producing taxa depletion in the gut microbiota of glycogen storage disease type 1 patients
topic glycogen storage disease
GSD
diet
gut microbiota
short-chain fatty acids
inflammation
url https://www.mdpi.com/2218-1989/10/4/133
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