BIOCHEMICAL AND GENETICAL CHARACTERISATION OF NOVEL ENZYMES INVOLVED IN RIBOFLAVIN METABOLISM. INVOLVMENTS IN RIBOFLAVIN-RESPONSIVE MYOPATHY

Pubblicazioni Scientifiche

• ELECTROPHORESIS

  Coordinated and reversible reduction of enzymes involved in terminal oxidative metabolism in skeletal muscle mitochondria from a riboflavin-responsive, multiple acyl-CoA dehydrogenase deficiency patient

• EUROPEAN JOURNAL OF BIOCHEMISTRY

  A protein factor of rat liver mitochondrial matrix involved in flavinylation of dimethylglycine dehydrogenase

• EUROPEAN JOURNAL OF BIOCHEMISTRY

  The riboflavin/FAD cycle in rat liver mitochondria

• FEBS LETTERS

  Flavinylation of the precursor of mitochondrial dimethylglycine dehydrogenase by intact and solubilised mitochondria

• JOURNAL OF BIOLOGICAL CHEMISTRY

  Riboflavin uptake and FAD synthesis in Saccharomyces cerevisiae mitochondria -: Involvement of the Flx1p carrier in FAD export

• JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

  Decreased fatty acid β-oxidation in riboflavin-responsive, multiple acylcoenzyme A dehydrogenase-deficient patients is associated with an increase in uncoupling protein-3

• PROTEIN EXPRESSION AND PURIFICATION

  Over-expression in Escherichia coli, functional characterization and refolding of rat dimethylglycine dehydrogenase