Binding of human low density lipoproteins (LDL) to their specific receptor on cultured cells can be inhibited by treatment with 1,2-cyclohexanedione which blocks a number of functionally significant arginyl residues on the apolipoprotein. We have used this observation to examine the role of the receptor pathway in LDL catabolism in man. The plasma clearance rates of 125I-LDL and 131I-cyclohexan...

A series of 2-(l-alkylamino)vinylidene-l,3-cyclohexanedione was synthesized and assayed as inhibitors of the Hill reaction in isolated chloroplasts. The pls() value of this series was dramatical­ ly changed by modification of the peripheral of its hydrophilic moiety and the maximum p l50 value of this series was almost equal to that of DCMU. The results suggest that this series may be a good pr...

A series of new hexahydroquinoline and 1,4-dihydropyrimidine derivatives were synthesized. Condensation of 2-methyl- thiazole-4-carboxaldehyde (1) with 1,3-cyclohexanedione and alkyl 3- aminocrotonate afforded 4-(2-methyl-thiazol-4-yl)-hexahydroquinoline while condensation of aldehyde (1) with benzoyl acetone and thiourea gave 1,4-dihydropyrimidine derivatives. The stereochemistry of 1,4-dihydr...

The density functionals B3LYP, B3PW91, M062X, and CAM-B3LYP with the 6-311+G(d,p) basis set predict the cysteine sulfenic acid O-sulfenylation of the s-cis-ketoenol tautomer of 1,3-cyclohexanedione proceeds through a cyclic 14-membered transition state structure containing three water molecules.

In a heretofore-unreported reaction, 1,2-cyclohexandione reacts with a variety of alcohols under mild acid-catalyzed dehydration conditions to afford not only ethers of 1,2cyclohexanedione but the corresponding aryl ethers as well in moderate to good yield.

We report a case of Goldenhar syndrome and hereditary tyrosinemia type 1 (HTT1), to our knowledge an association not previously described. This case further increases the diversity of observations and clinical descriptions of patients with this complex syndrome. We discuss pathogenetic aspects, and demonstrate further evidence of the effectiveness of 2-(2-nitro-4-trifluoromethyl benzoyl)-1,3-cy...

Neurometabolic disorders such as tyrosinemia type 1 (TYRSN1) may interfere with brain metabolism and show symptoms of attention-deficit hyperactivity disorder (ADHD) in patients treated the enzyme inhibitor nitisinone [2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione, NTBC]. It has been reported that ADHD treatment improves compliance, which is imperative for long-term prognosis TYRSN1...

Kringle 4, an elastase fragment of plasminogen which carries one of the homologous o-aminocarboxylic acidbinding sites of the protein, has been subjected to chemical modification to identify the residues essential for ligand binding. Modification of kringle 4 with 1,2-cyclohexanedione or l-ethyl-3-(3-dimethylaminopropyl)carbodiimide has been found to abolish ligand binding as detected by the lo...