Unraveling the drivers and consequences of gut microbiota disruption in Fabry disease: the lyso-Gb3 link
Fabry disease is characterized by lyso-Gb3 accumulation, gastrointestinal symptoms and severe nephropathy and myocardiopathy. Clinically relevant lyso-Gb3 concentrations disrupt the gut microbiota homeostasis. Biofilm formation by enteric bacteria is modulated, favoring Bacteroides fragilis growth. In complex gut bacterial communities, lyso-Gb3 reduced bacteria associated with a healthy microbiota (Bifidobacterium, Akkermansia, Lactobacillus, and butyrate-producing Blautia coccoides-Eubacterium rectale, and Clostridium leptum) and butyrate production. We now discuss the clinical and pathophysiological implications of these findings as butyrate has anti-inflammatory properties through epigenetic regulation of histone acetylation that may protect against nephropathy and myocardiopathy. Lyso-Gb3 modulation of the gut microbiota may directly contribute to the gastrointestinal symptoms and indirectly to systemic manifestations of Fabry disease through modulation of the epigenetic regulation of inflammation.
| Main Authors: | , , , , , |
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| Other Authors: | |
| Format: | artículo biblioteca |
| Language: | English |
| Published: |
Future Medicine
2020
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| Online Access: | http://hdl.handle.net/10261/220238 http://dx.doi.org/10.13039/501100003329 http://dx.doi.org/10.13039/100012818 http://dx.doi.org/10.13039/501100000780 http://dx.doi.org/10.13039/501100004587 |
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