2019
DOI: 10.1038/s41598-019-48426-4
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Lyso-Gb3 modulates the gut microbiota and decreases butyrate production

Abstract: Fabry disease is a rare X-linked lysosomal storage disorder resulting from deficient activity of α-galactosidase A, leading to the accumulation of glycosphingolipids such as globotriaosylsphingosine (lyso-Gb3). The gastrointestinal symptoms of this disease may be disabling, and the life expectancy of affected patients is shortened by kidney and heart disease. Our hypothesis was that lyso-Gb3 may modify the gut microbiota. The impact of a clinically relevant concentration of lyso-Gb3 on mono- or multispecies ba… Show more

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Cited by 27 publications
(32 citation statements)
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“…It is speculated that the striking reduction of butyrate releases the break on histone deacetylase inhibition, allowing for increased inflammation. Concomitantly this may trigger induction of GB3 synthase, thus more lyso-GB3 formation, and may worsen kidney disease and heart disease [ 83 ].…”
Section: Globotriaosylsphingosine/lyso-gb3 (Fabry Disease)mentioning
confidence: 99%
“…It is speculated that the striking reduction of butyrate releases the break on histone deacetylase inhibition, allowing for increased inflammation. Concomitantly this may trigger induction of GB3 synthase, thus more lyso-GB3 formation, and may worsen kidney disease and heart disease [ 83 ].…”
Section: Globotriaosylsphingosine/lyso-gb3 (Fabry Disease)mentioning
confidence: 99%
“…Although there is little information on microbiota and chronodisruption in CKD, both obesity and metabolic syndrome increase the risk of CKD. Conversely, CKD has been associated with altered microbiota patterns and metabolites accumulated in CKD may modulate the gut microbiota and butyrate production [62][63][64].…”
Section: Dietary Cluesmentioning
confidence: 99%
“…A remarkable decrease in butyrate production of gut microbiota is usually associated with certain functional disorders. For example, Fabry disease is often attributed to the accumulation of globotriaosylsphingosine (lyso-Gb3) which alters the formation of SCFAs, resulting in a significant reduction in butyrate concentration [53]. The present study recorded the significant highest fecal butyrate concentration ( P < 0.05) in FCRPG, and the second highest value ( P < 0.05) was observed in CRPG, which means that dietary supplemented with FCRP or CRP can evidently increase epithelial energy supply and maintain normal celluar proliferation and differentiation.…”
Section: Discussionmentioning
confidence: 99%