ketoaciduria

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Chemistry of free radicals produced by oxidation of endogenous α-aminoketones. A study of 5-aminolevulinic acid and α-aminoacetone by fast kinetics spectroscopy.

Morlière P1, Hug GL2, Patterson LK3, Mazière JC4, Ausseil J4, Dupas JL5, Ducroix JP6, Santus R7, Filipe P8.
Biochimica et biophysica acta.Biochim Biophys Acta.2014 Oct;1840(10):3190-7. doi: 10.1016/j.bbagen.2014.07.002. Epub 2014 Jul 10.
BACKGROUND: Excess 5-aminolevulinic acid (ALA) and α-aminoacetone (AA) are implicated in ketosis, porphyrinpathies and diabetes. Pathologic manifestations involve O2(-), H2O2, OH, enoyl radicals (ALA and AA) and their oxidation end products.METHODS: To characterize enoyl radicals resulting from rea
PMID 25018004

A comparative study of the metabolic profile, insulin sensitivity and inflammatory response between organically and conventionally managed dairy cattle during the periparturient period.

Abuelo A, Hernández J, Benedito JL, Castillo C.
Animal : an international journal of animal bioscience.Animal.2014 Sep;8(9):1516-25. doi: 10.1017/S1751731114001311. Epub 2014 Jun 11.
The number of organically managed cattle (OMC) within the European Union has increased tremendously in the last decade. However, there are still some concerns about animals under this farming system meeting their dietary requirements for milk production. The aim of this study was to compare the meta
PMID 24916777

Acute metabolic acidosis in a GLUT2-deficient patient with Fanconi-Bickel syndrome: new pathophysiology insights.

Mihout F1, Devuyst O2, Bensman A3, Brocheriou I4, Ridel C5, Wagner CA2, Mohebbi N2, Boffa JJ6, Plaisier E6, Ronco P6.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.Nephrol Dial Transplant.2014 Sep;29 Suppl 4:iv113-iv116. doi: 10.1093/ndt/gfu018.
Fanconi-Bickel syndrome is a rare autosomal-recessive disorder caused by mutations in the SLC2A2 gene coding for the glucose transporter protein 2 (GLUT2). Major manifestations include hepatomegaly, glucose intolerance, post-prandial hypoglycaemia and renal disease that usually presents as proximal
PMID 25165176