Glutaric acid (GA) has been implicated in the mechanism of neurodegeneration in glutaric aciduria type I. In the present study, the potential cytotoxic effects of GA (0.1~50 mM for 24~96 h) were examined in cultured primary rat striatal neurons. Results showed increase in the number of cells labeled by annexin-V or with apoptotic features shown by Hoechst/PI staining and transmission electron m...

Binary heterogeneous nucleation of watersuccinic/glutaric/malonic/adipic acid on nanometer-sized particles is investigated within the frame of classical heterogeneous nucleation theory. Homogeneous nucleation is also included for comparison. It is found that the nucleation probabilities depend on the contact angle and on the size of the seed particles. New thermodynamical properties, such as sa...

The aim of the present study was to investigate the clinical, biochemical and genetic mutation characteristics of two cases of late-onset glutaric aciduria type I (GA-I) in Uighur. The clinical data and glutaryl-CoA dehydrogenase (GCDH) genetic test results of two cases of late-onset GA-I in Uighur were collected and analyzed, and reviewed with relevant literature. One patient with late-onset G...

Case presentation: Male patient, 6 months old, admitted due to seizure associated with severe refractory hypoglycemia. Patient had been experiencing unusual sleepiness for 8 days, and vomiting after feedings. Brain CT showed prominence of the bilateral frontotemporal extra-axial space Sylvian fissures, EEG revealed a slow diffuse moderate disturbance background activity MRI extensive areas diff...

INTRODUCTION: Glutaric Acidemia type I (GA-1) is an inborn error of lysine, hydroxylysine and tryptophan catabolism. Due to deficiency in glutarylCoA dehydrogenase (Gcdh), these amino acids can not be completely catabolized and lead to accumulation of glutaric acid in the brain, blood and urine. The world wide prevalence is approximately 1:30,000. The common findings at autopsy are severe neuro...

H NMR and C NMR spectrometry (1-dimensional and 2-dimensional) have been used to assign chemical resonances and determine the degrees of branching for polyesters synthesized by the Lewis acid (dibutyltin(IV)oxide)-catalyzed polycondensation of glycerol with either succinic acid (n (aliphatic chain length) = 2), glutaric acid (n = 3) or azelaic acid (n = 7) in quasi-melt solutions with toluene. ...

Glutaric acidemia type 1 (GA1) is an inborn error of metabolism caused by the deficiency enzyme glutaryl-CoA dehydrogenase, with consequent accumulation aminoacids lysine, hydroxylsine and tryptophan. About in every 100,000 individuals are affected disease. Neurological manifestations variable include acute chronic encephalopathic crises, dystonia, motor cognitive deficits, as well neuroimaging...

In pursuit of understanding structure–property relationships for the melting point depression binary eutectic mixtures, influence anion on solid–liquid (S–L) phase behavior was explored mixtures glutaric acid + tetraethylammonium chloride, bromide, and iodide. A detailed experimental evaluation S–L revealed that is shifted toward lower temperatures higher salt contents upon decreasing ionic rad...

Nitrones undergo dynamic exchange in chloroform at room temperature through two mechanisms-hydrolysis and recombination or hydroxylamine addition/elimination; this dynamic exchange is harnessed to select a nitrone-based bis(amidopyridine) receptor for diacids from a group of four nitrones through its binding to a glutaric acid-based target.