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Category: Heterocycles, Isotope Labelled Compounds /// Application Notes: 4,5-Dimethyl-3-hydroxy-2(5H)-furanone-13C2 is a labelled analogue of 4,5-Dimethyl-3-hydroxy-2(5H)-furanone (D473600). 4,5-Dimethyl-3-hydroxy-2(5H)-furanone is a fragrant compound that is a key indicator of maple syrup urine disease, a disorder affecting newborn children that is the result of its inability to properly metabolize Valine (V094205), Leucine (L330110) and Isoleucine (I820175). 4,5-Dimethyl-3-hydroxy-2(5H)-furanone is naturally found in fenugreek seeds, and is an odourant present in sherry wines. /// References: Blank, I., et al.: Prog. Flav. Prec. Stud., No vol. given, 103 (1993)| Guerra, P. & Yaylayan, V.: J. Agr. Food Chem., 59, 4699 (2011)| Mackenzie, D. & Woolf, L.: Brit. Med. J., 1, 90 (1959)| Podebrad, F., et al.: J. Inh. Metab. Dis., 22, 107 (1999)