Articles with "hydroxyisobutyric acid" as a keyword



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3‐Hydroxyisobutyric acid dehydrogenase deficiency: Expanding the clinical spectrum and quantitation of D‐ and L‐3‐Hydroxyisobutyric acid by an LC–MS/MS method

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Published in 2022 at "Journal of Inherited Metabolic Disease"

DOI: 10.1002/jimd.12486

Abstract: A deficiency of 3‐hydroxyisobutyric acid dehydrogenase (HIBADH) has been recently identified as a cause of primary 3‐hydroxyisobutyric aciduria in two siblings; the only previously recognized primary cause had been a deficiency of methylmalonic semialdehyde dehydrogenase,… read more here.

Keywords: hydroxyisobutyric acid; acid dehydrogenase; acid; clinical spectrum ... See more keywords
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2-hydroxyisobutyric acid (2-HIBA) modulates ageing and fat deposition in Caenorhabditis elegans

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Published in 2022 at "Frontiers in Molecular Biosciences"

DOI: 10.3389/fmolb.2022.986022

Abstract: High levels of 2-hydroxyisobutyric acid (2-HIBA) were found in urines of patients with obesity and hepatic steatosis, suggesting a potential involvement of this metabolite in clinical conditions. The gut microbial origin of 2-HIBA was hypothesized,… read more here.

Keywords: hiba; hydroxyisobutyric acid; acid hiba; caenorhabditis elegans ... See more keywords