Using the murine model of tyrosinemia type 1 (fumarylacetoacetate hydrolase [FAH] deficiency; FAH⁻/⁻ mice) as a paradigm for orphan disorders, such as hereditary metabolic liver diseases, we evaluated fibroblast-derived FAH⁻/⁻-induced pluripotent stem cells (iPS cells) as targets for gene correction in combination with the tetraploid embryo complementation method. First, after characterizing th...

using hartree–fock (hf) and ِِdensity functional theory (dft) calculations the thermodynamic properties such as thermal energy , , thermal enthalpy , , thermal entropy , , thermal gibbs free energy , , heat capacity ,cv, and molecular structures of several species involving in keto↔enol tautomerism related to acetaldehyde (a), 5,5-dimethyl-1,3-cyclohexanedione (dimedone) and  acetylacetone (aa) h...

Since its discovery almost 40 years ago, S-hydroxylation ( SOH) of cysteine thiol side chains at active and allosteric sites within proteins has emerged as a central post-translational modification. At present, more than 200 transcription factors, signaling proteins, metabolic enzymes, proteostasis regulators, and cytoskeletal components that undergo sulfenic acid modification have been identif...

The binding of low-density lipoproteins (LDL) as well as LDL modified by cyclohexanedione (CHD-LDL) to gel-filtered platelets (GFP) and its effect on platelet function were studied in normal and in homozygous familial hypercholesterolaemic (HFH) subjects. Only normal-derived LDL could significantly compete with normal 125I-labelled LDL for binding to normal platelets. When GFP from normal subje...

The suitability of seven media for the enumeration of Listeria spp. was evaluated at 30 degrees C for 48 h. The media tested were (i) the original McBride Listeria agar formulation (with glycine); (ii) modified McBride agar containing glycine anhydride; (iii) LiCl-phenylethanol-moxalactam (LPM) agar; (iv) acriflavine-ceftazidime agar; (v) Rodriguez isolation agar (RISA); (vi) modified Vogel-Joh...

BACKGROUND Hereditary Tyrosinemia type 1 (HT1) is a rare metabolic disorder caused by a defect in the enzyme Fumarylacetoacetate Hydrolase. Due to this defect, toxic products accumulate which, in turn, cause liver and kidney dysfunction. Treatment with 2-(2-nitro-4-trifluoromethylbenoyl)-1,3-cyclohexanedione (NTBC) and diet has diminished these problems, but recent data indicate that HT1 patien...

INTRODUCTION Hereditary Tyrosinemia type 1 (HT1) is a rare metabolic disease caused by a defect in the tyrosine degradation pathway. Current treatment consists of 2-(2-nitro-4-trifluoromethylbenoyl)-1,3-cyclohexanedione (NTBC) and a tyrosine and phenylalanine restricted diet. Recently, neuropsychological deficits have been seen in HT1 patients. These deficits are possibly associated with low bl...

The effects o f individual or combined treatment o f the cyclohexanedione herbicide sethoxy­ dim and the safener dichlormid on total lipid synthesis, protein synthesis and acetyl-CoA car­ boxylase (ACCase, EC 6.4.1.12) activity o f grain sorghum [Sorghum bicolor (L.) M oench, var. G623] were investigated. Sethoxydim and dichlormid were tested at concentrations o f 0, 5, 50, and 100 |iM each. Se...

Technical iodine was found to catalyze the condensation between 2-aminobenzohydrazide (1) and some aldehydes and ketones in absolute ethanol under mild conditions to afford hydrazone and quinazoline derivatives, respectively. Condensation of 1 with terephthalaldehyde (2) in 1 : 1 molar ratios afforded the hydrazone 3, while hydrazone 4 was formed on using a double molar ratio of 1. On the other...