Liver Metabolomic Profiling Reveals Distinct Signatures Between Steatosis and Metabolic Dysfunction-Associated Steatohepatitis. [PDF]
Liver IntABSTRACT Background and Aims Metabolic dysfunction‐associated steatotic liver disease (MASLD), the most common chronic liver disease worldwide, is closely linked to obesity and metabolic syndrome. The reason some patients with MASLD develop metabolic dysfunction‐associated steatohepatitis (MASH) and which metabolic changes in the liver are linked to ...
Palomurto S +14 more
europepmc +2 more sources
Structural characterisation of nucleotide sugar short-chain dehydrogenases/reductases from the thermophilic pseudomurein-containing methanogen Methanothermobacter thermautotrophicus ΔH. [PDF]
FEBS JNucleotide sugar short‐chain dehydrogenases/reductases (NS‐SDRs) are involved in pseudomurein and capsular polysaccharide formation in methanogenic Archaea. Methanothermobacter thermautotrophicus possesses several NS‐SDRs labelled Mth375, Mth380, Mth373, Mth631 and Mth1789.
Carbone V +4 more
europepmc +2 more sources
Galactose Epimerase Deficiency: Expanding the Phenotype [PDF]
, 2017 Galactose epimerase deficiency is an inborn error of metabolism due to uridine diphosphate-galactose-4'-epimerase (GALE) deficiency. We report the clinical presentation, genetic and biochemical studies in two siblings with generalized GALE deficiency ...
A Alano +14 more
core +1 more source
Metabolic engineering of microbes for oligosaccharide and polysaccharide synthesis [PDF]
, 2006 Metabolic engineering has recently been embraced as an effective tool for developing whole-cell biocatalysts for oligosaccharide and polysaccharide synthesis.
Anne Ruffing, Rachel Chen
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Journal of Lipid Research, 1976 An enzyme that transfers galactose from UDP-Gal to ganglioside GM2 (Tay-Sachs ganglioside) was concentrated 50 times in Golgi apparatus from rat liver relative to total homogenates.
F E Wilkinson, D J Morré, T W Keenan
doaj +1 more source
Functional analysis of the Lactococcus lactis galU and galE genes and their impact on sugar nucleotide and exopolysaccharide biosynthesis [PDF]
, 2001 We studied the UDP-glucose pyrophosphorylase (galU) and UDP-galactose epimerase (galE) genes of Lactococcus lactis MG1363 to investigate their involvement in biosynthesis of UDP-glucose and UDP-galactose, which are precursors of glucose- and galactose ...
Boels, I.C. +3 more
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Orphanet Journal of Rare Diseases, 2018 Background Clinical outcome of patients with a classical presentation of galactosemia (classical patients) varies substantially, even between patients with the same genotype.
Michel van Weeghel +5 more
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Urinary reducing substances in neonatal intrahepatic cholestasis caused by citrin deficiency
Journal of Pediatric and Neonatal Individualized Medicine, 2014 Neonatal cholestasis due to citrin deficiency is an autosomal recessive metabolic disorder caused by mutations in SLC25A13 gene. Mutations in this gene have a relatively high prevalence in East-Asian races compared to European or Afro-Caribbean races ...
Ajmal Kader +4 more
doaj +1 more source
Disease Models & Mechanisms, 2014 Classic galactosemia (CG) is an autosomal recessive disorder resulting from loss of galactose-1-phosphate uridyltransferase (GALT), which catalyzes conversion of galactose-1-phosphate and uridine diphosphate (UDP)-glucose to glucose-1-phosphate and UDP ...
Patricia Jumbo-Lucioni +2 more
doaj +1 more source
Genome-wide analysis of the UDP-glucose dehydrogenase gene family in Arabidopsis, a key enzyme for matrix polysaccharides in cell walls [PDF]
, 2007 Arabidopsis cell walls contain large amounts of pectins and hemicelluloses, which are predominantly synthesized via the common precursor UDP-glucuronic acid.
Klinghammer, Michaela, Tenhaken, Raimund
core +1 more source