Aerobic growth of Rhodococcus aetherivorans BCP1 using selected naphthenic acids as the sole carbon and energy sources [PDF]
, 2018 Naphthenic acids (NAs) are an important group of toxic organic compounds naturally occurring in hydrocarbon deposits. This work shows that Rhodococcus aetherivorans BCP1 cells not only utilize a mixture of eight different NAs (8XNAs) for growth but they ...
Cappelletti M. +12 more
core +2 more sources
The Function of Arg-94 in the Oxidation and Decarboxylation of Glutaryl-CoA by Human Glutaryl-CoA Dehydrogenase [PDF]
Journal of Biological Chemistry, 2001 Glutaryl-CoA dehydrogenase catalyzes the oxidation and decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2). Inherited defects in the protein cause glutaric acidemia type I, a fatal neurologic disease. Glutaryl-CoA dehydrogenase is the only member of the acyl-CoA dehydrogenase family with a cationic residue, Arg-94, situated in the binding site of
T M, Dwyer +4 more
openaire +2 more sources
Cholesterol metabolism is altered in Rett syndrome: A study on plasma and primary cultured fibroblasts derived from patients [PDF]
, 2014 Rett (RTT) syndrome is a severe neurological disorder that affects almost exclusively females. Several detectable mutations in the X-linked methyl-CpG-binding protein 2 gene (MECP2) are responsible for the onset of the disease.
Di Tunno, Ilenia +6 more
core +9 more sources
ACSS2 involved in acetyl-CoA synthesis regulates skeletal muscle function. [PDF]
FEBS LettThe enzyme acyl‐coenzyme A synthetase short‐chain family member‐2 (ACSS2) catalyzes the conversion of acetate to acetyl‐CoA, but its function in skeletal muscle is unclear. We studied ACSS2 deficiency in mouse and fly models. Skeletal muscle from the mouse model showed atrophic fibers, excess lipid, and depleted NADH.
Gunasekaran M +6 more
europepmc +2 more sources
Relative expression of genes of terpene metabolism in different tissues of Artemisia annua L [PDF]
, 2011 BackgroundRecently, Artemisia annua L. (annual or sweet wormwood) has received increasing attention due to the fact that the plant produces the sesquiterpenoid endoperoxide artemisinin, which today is widely used for treatment of malaria.
Linda Olofsson +3 more
core +1 more source
A Treatable Neurometabolic Disorder: Glutaric Aciduria Type 1
Case Reports in Pediatrics, 2014 Glutaric aciduria type 1 (GA-1) is an autosomal recessive disorder of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-CoA dehydrogenase. It results in the accumulation of 3-hydroxyglutaric and glutaric acid.
S. Pusti, N. Das, K. Nayek, S. Biswas
doaj +1 more source
The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neurons [PDF]
, 2006 Multi-objective evolutionary algorithms (MOEAs) have become increasingly popular as multi-objective problem solving techniques. Most studies of MOEAs are empirical. Only recently, a few theoretical results have appeared.
Katja Brauburger +2 more
core +3 more sources
Occurrence of subdural hematomas in Dutch glutaric aciduria type 1 patients [PDF]
, 2016 Patients with glutaric aciduria type 1 (GA1), a rare inherited metabolic disorder, have an increased risk for subdural hematomas (SDHs). GA1 is therefore generally included in the differential diagnosis of children presenting with SDHs.
Rijn, R.R. (Rick) van +5 more
core +1 more source
Glutaric aciduria type I with high residual glutaryl‐CoA dehydrogenase activity [PDF]
Developmental Medicine & Child Neurology, 1998 Two brothers with dystonia and slight MRI changes in the basal ganglia had normal urinary glutaric acid excretion, but slightly increased 3‐hydioxyglutarate and conjugated glutarate excretions. Both siblings have high residual glutaryl‐CoA dehydrogenase activity, and are compound heterozygotes for two mutations ‐ R227P and V400M ‐ reported to be ...
M, Pineda +5 more
openaire +2 more sources
Dietary moderately oxidized oil induces expression of fibroblast growth factor 21 in the liver of pigs [PDF]
, 2012 BACKGROUND: Fibroblast growth factor 21 (FGF21), whose expression is induced by peroxisome proliferator-activated receptor alpha (PPARalpha), has been recently identified as a novel metabolic regulator which plays a crucial role in glucose homeostasis ...
Eder, Klaus +2 more
core +2 more sources