Glutaric aciduria type I (GA I) is an autosomal recessive disorder caused by a deficiency of glutaryl-CoA dehydrogenase. Although over 400 patients confirmed as GA I have been reported, reports from the Asian population had contributed to the minor proportion. We recently diagnosed two cases of GA I confirmed with mutational analysis. Here, we present their rather atypical clinical presentation...

Coriandrum sativum (Coriander) has been documented as a traditional treatment for cholesterol and diabetes patients. In the present study, coriander seeds incorporated into diet and the effect of the administration of coriander seeds on the metabolism of lipids was studied in rats, fed with high fat diet and added cholesterol. The seeds had a significant hypolipidemic action. In the experimenta...

Statin drugs have previously been shown to very significantly reduce cardiovascular disease events in a number of large clinical trials.1 As a result, statins are now considered to represent one of the most powerful classes of agents for the treatment of cardiovascular diseases.2 Statins are rapidly becoming frontline therapy for diabetes mellitus, hypertension, and other known cardiovascular d...

We demonstrate that the 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase inhibitors atorvastatin and simvastatin enhance functional outcome and induce brain plasticity when administered after stroke to rats. With atorvastatin treatment initiated 1 day after stroke, animals exhibited significant increases in vascular endothelial growth factor, cyclic guanosine monophosphate, angiogenes...

The effects of carboxyacyl phosphatidylethanolamine derivatives (CA-PE) on the physicochemical properties of liposomes were examined from standpoints of zeta-potential, agglutination, phase transition temperature, microviscosity and micropolarity. Absolute zeta-potential of liposomes containing Succinyl-PE or Glutaryl-PE exceeded that of those containing Dodecanyl-PE. The addition of Succinyl-P...

The clinical use of statins as therapeutic tools for osteoporosis has not yet reached the status of solid scientific dogma, even though it has been almost 15 years since the emergence of the first experimental evidence on the effect of this class of drugs on bone metabolism, specifically stimulating the formation of “new bone”. Statins are a groupof competitive inhibitors consistingof the hydro...

The mechanisms underlying ethylmalonic-adipic aciduria were studied in a 5-yr-old girl. Oxidation of radioactive substrates by cultured skin fibroblasts from the proband and asymptomatic family members was also determined and compared to that by normal fibroblasts and that by cells from a patient with glutaric aciduria type II. Feeding medium-chain triglycerides promptly induced vomiting and le...

Glutaric aciduria type 1 (GA1) is a rare, inherited mitochondrial disorder that results from deficiency of mitochondrial glutaryl-CoA dehydrogenase. Most patients develop neurological dysfunction early in life, which leads to severe disabilities. We present a 37-month-old girl with GA1 manifested as macrocephaly and hypotonia who received comprehensive dental restoration surgery under general a...

Efficient delivery of short interfering RNAs reflects a prerequisite for the development of RNA interference therapeutics. Here, we describe highly specific nanoparticles, based on near infrared fluorescent polymethine dye-derived targeting moieties coupled to biodegradable polymers. The fluorescent dye, even when coupled to a nanoparticle, mimics a ligand for hepatic parenchymal uptake transpo...

It is well known that statins exert their main effect by inhibiting cholesterol synthesis through the inhibition of the 3-hydroxy-3-methyl-glutaryl-CoA reductase enzyme. The pleiotropic effects of statins, which are independent of their inhibition of cholesterol synthesis, have explained many of the beneficial effects of these drugs in a variety of disorders such as malignancies, infection, and...