Results 41 to 50 of about 88 (85)

Glutaric aciduria mediated by gut bacteria

Journal of Inherited Metabolic Disease, 1995
Item does not contain ...
Wendel, U.A.H., Bakkeren, J.A.J., Jong, J.G.N. de, Bongaerts, G.P.A.   +3 more
openaire   +6 more sources

Recent Progress in Understanding Glutaric Acidemias

Enzyme, 1987
Glutaric acidemia, which is due to inherited deficiency of glutaryl-CoA dehydrogenase, is characterized clinically by progressive dystonia and dyskinesia in childhood, and pathologically by degeneration of the caudate and putamen. Results using newer imaging techniques (computer tomography and magnetic resonance image scanning) suggest that ...
James P. Loehr, Stephen I. Goodman, Frank E. Frerman   +2 more
openaire   +3 more sources

Hydrolysis of glutaric anhydride to glutaric acid in the presence of β-cyclodextrin. Crystallographic and NMR study [PDF]

Journal of Inclusion Phenomena and Macrocyclic Chemistry, 2010
Crystallization of glutaric anhydride in the presence of β-cyclodextrin (βCD) from aqueous solution resulted in crystals of the glutaric acid/βCD inclusion complex. The result was verified by NMR spectroscopic experiments, which moreover showed that βCD does not protect glutaric anhydride from hydrolysis.
Konstantina Yannakopoulou, Irene M. Mavridis, Anastasia Paulidou   +2 more
openaire   +1 more source

Neuropathology in glutaric acidaemia type 1

Acta Neuropathologica, 1988
The neuropathology in three cases of glutaric acidaemia type 1 is presented. All three showed extensive neuronal loss in the caudate nucleus and the putamen, with only small numbers of large neurons surviving. The globus pallidus showed moderate shrinkage and gliosis but no conspicuous decreases in neurons.
Anderson Rm, David M. Danks, G Wise, James McGill, Eric Haan, Stephen I. Goodman, C. W. Chow, Evans Wa, Bette K. Kleinschmidt-DeMasters   +8 more
openaire   +3 more sources

Structure of sodium hydrogen glutarate dihydrate [PDF]

Acta Crystallographica Section C Crystal Structure Communications, 1991
C 5 H 7 O 4 − •Na + •2H 2 O cristallise dans P21/c avec a=5,371, b=13,367, c=11,587 A, β=93,72°, Z=4; affinement jusqu'a R=0.034. La structure contient des tres courtes liaisons hydrogene asymetriques qui lient les residus de l'acide glutarique en chaines infinies. Les ions Na + de coordination 6 lies par des molecules d'eau sont arranges en colonnes
Macdonald, A.L., Morrison, P. D., Murray, A., Freer, A.A.   +3 more
openaire   +2 more sources

Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and β-Hydroxyglutarate: Explanation of the symptoms in glutaric aciduria?

Clinica Chimica Acta, 1976
Glutaric aciduria is a disorcer of lysine, tryptophan, and hydroxylysine metabolism characterized by intermittent metabolic acidemia, dystonia, athetosis and mental retardation. It is due to a recessively inherited deficiency of glutaryl-CoA dehydrogeanse, the enzyme(s) which catalyze the dehydrogenation of glutaryl-CoA to glutaconyl-CoA and ...
Paul G. Moe, Stephen I. Goodman, Oddvar Stokke   +2 more
openaire   +3 more sources

On the neurotoxicity of glutaric, 3‐hydroxyglutaric, and trans‐glutaconic acids in glutaric acidemia type 1

Journal of Neuroscience Research, 2004
AbstractGlutaric acidemia type 1 (GA1) is an autosomal recessively inherited deficiency of glutaryl‐CoA dehydrogenase. Accumulating metabolites, 3‐hydroxyglutaric (3‐OH‐GA), glutaric (GA), and trans‐glutaconic (TG) acids, have been proposed to be involved in the development of the striatal degeneration seen in children with GA1 via an excitotoxic ...
Lund, Trine Meldgaard, Christensen, E, Kristensen, A S, Schousboe, A, Lund, A M   +4 more
openaire   +4 more sources

Glutaric aciduria types I and II

Brain and Development, 2006
Glutaric aciduria type I is an autosomal recessive disorder resulting from a deficiency of glutaryl-CoA dehydrogenase. This leads to an accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitine deficiency. The symptomatology is discussed, especially those resulting from lesions in the basal ganglia, and the encephalopathic episodes ...
openaire   +3 more sources

2-(2,5-Dichlorophenylhydrazono)glutaric Acid and 2-(3,5-Dichlorophenylhydrazono)glutaric Acid

Acta Crystallographica Section C Crystal Structure Communications, 1996
The title compounds, C11H10Cl2N2O4, are constitutional isomers which exhibit similar molecular geometry. The configuration about the N=C bond is E in both molecules. The same packing pattern was found in both structures. Hydrogen-bonded dimers, formed around the inversion centres via O-H ...
Snježana Antolić, Jerry D. Cohen, Biserka Kojić-Prodić   +2 more
openaire   +3 more sources

Glutaric aciduria: Biochemical and morphologic considerations

The Journal of Pediatrics, 1977
Biochemical and morphologic studies on a patient with glutaric aciduria are presented. Generalized aminoaciduria, alpha-aminoadipic aciduria, and saccharopinuria were noted just prior to death, as well as glutaconic aciduria greater than beta-hydroxyglutaric aciduria.
Paul G. Moe, Paul G. Moe, Diana J. Breslich, Diana J. Breslich, Robert H. Shikes, Robert H. Shikes, Michael D. Norenberg, Michael D. Norenberg, Stephen I. Goodman, Stephen I. Goodman   +9 more
openaire   +3 more sources