Background Maple Syrup Urine Disease (MSUD) is a rare autosomal recessive metabolic error, characterized by Branched Chain α-Keto-acid Dehydrogenase Complex (BCKDC) deficiency. Mutations in 3 genes can lead to abnormal metabolism and accumulation of leucine, isoleucine, valine and corresponding keto-acids. MSUD affects 1 in 185,000 infants globally. Seizure is a common presentation among neonat...

The title mol-ecule, C(20)H(24)O(2), is a chalconoid derivative in which the keto-enone group is slightly distorted from planarity; the O=C-C=C torsion angle is 12.24 (13)°.

Introduction Conclusions References

Keto acids and reduced-oxygen-scavenging enzymes were examined for their roles in supporting the growth of Legionella species and for their potential reactions between the chemical components of the media. When grown in an experimental ACES (2-[(2-amino-2-oxoethyl)-amino] ethanesulfonic acid)-buffered chemically defined (ABCD) broth, the presence of keto acids shortened the lag periods, increas...

L-Methionine sulfoximine is a substrate of L-amino acid oxidase (Crotalus adamanteus), glutamine transaminase, and y-cystathionase. In the reaction catalyzed by L-amino acid oxidase, methionine sulfoximine is converted to oc-imino-y-methylsulfoximinylbutyrate, which undergoes rapid y elimination yielding methane sulfinimide and 2-imino-3-butenoic acid. Methane sulfinimide is converted to methan...

The present paper describes a new enzyme from animal tissues which catalyzes the keto-enol tautomeriaation of the phenylpyruvic acids. Preliminary accounts of this tautomerase have been published (1, 2). The enzyme has not yet proved to be essential in any biological reaction, but a physiological role may be expected since specific tautomers of a-keto acids are substrates or products in some re...

The purpose of this study was to elucidate the mechanism by which acetylcholine (ACh) promotes prostacyclin (PGI2) production in cultured coronary endothelial cells (CEC) of the rabbit heart. ACh-induced production of PGI2, measured as immunoreactive 6-keto-PGF1alpha, was enhanced by increasing the extracellular calcium (Ca++) concentration and reduced by Ca++ depletion. The receptor-operated C...

Hyperoxia induces skin vasoconstriction in humans, but the mechanism is still unclear. In the present study we examined whether the vasoconstrictor response to hyperoxia is through activated adrenergic function (protocol 1) or through inhibitory effects on nitric oxide synthase (NOS) and/or cyclooxygenase (COX) (protocol 2). We also tested whether any such vasoconstrictor effect is altered by b...

The behavior of hydroxypyruvic acid, CHzOH. CO. COOH, and some of its transformation products toward periodic acid, treated in detail in the preceding paper (I), suggested an orienting study of the action of this oxidant on glyoxylic acid, CHO. COOH, and other a-keto acids or on compounds giving rise to such keto acids, following oxidation by periodate. While cr-glycols, a-ketols, a-diketones, ...