Purification and Characterization of an Arene cis-Dihydrodiol Dehydrogenase Endowed with Broad Substrate Specificity toward Polycyclic Aromatic Hydrocarbon Dihydrodiols [PDF]
Initial reactions involved in the bacterial degradation of polycyclic aromatic hydrocarbons (PAHs) include a ring-dihydroxylation catalyzed by a dioxygenase and a subsequent oxidation of the dihydrodiol products by a dehydrogenase. In this study, the dihydrodiol dehydrogenase from the PAH-degrading Sphingomonas strain CHY-1 has been characterized.
arxiv
Assessment of urinary 6‐oxo‐pipecolic acid as a biomarker for ALDH7A1 deficiency
Abstract ALDH7A1 deficiency is an epileptic encephalopathy whose seizures respond to treatment with supraphysiological doses of pyridoxine. It arises as a result of damaging variants in ALDH7A1, a gene in the lysine catabolism pathway. α‐Aminoadipic semialdehyde (α‐AASA) and Δ1‐piperideine‐6‐carboxylate (P6C), which accumulate because of the block in ...
Youssef Khalil+9 more
wiley +1 more source
Abstract The classic plant growth‐promoting phytohormone cytokinin has been identified and established as a mediator of pathogen resistance in different plant species. However, the resistance effect of structurally different cytokinins appears to vary and may regulate diverse mechanisms to establish resistance.
Dominik K. Großkinsky+7 more
wiley +1 more source
Substrates and mechanism of 2-hydroxyglutaryl-CoA-dehydratase from Clostridium symbiosum [PDF]
Muconyl-CoA, 2-hydroxyadipoyl-CoA, oxalocrotonyl-CoA and butynedioyl- CoA were synthesised and characterised as substrates of the (R)-2- hydroxyglutaryl-CoA dehydratase from Clostridium symbiosum.
Parthasarathy, Anutthaman
core +1 more source
Glutaric aciduria Type I (GA-I) is a rare inherited metabolic disease, deficiency of glutaryl-CoA dehydrogenase results in accumulation of the putatively neurotoxic metabolites glutaric and 3-hydroxyglutaric acid (GA, 3-OH-GA) in body tissues ...
Ebru Canda+11 more
doaj +1 more source
Oxidative stress and cellular senescence: Roles in tumor progression and therapeutic opportunities
Cellular senescence is intrinsically related to multiple aspects of tumor development. Oxidative stress contributes to tumor development by regulating cellular senescence. Targeting cellular senescence by modulating oxidative stress can benefit tumor management.
Ping Jin+7 more
wiley +1 more source
Evidence of a single origin for the most frequent mutation (R402W) causing glutaryl-CoA dehydrogenase deficiency: Identification of 3 novel polymorphisms and haplotype definition [PDF]
Christiane Busquets+2 more
openalex +2 more sources
Treatment of glutaric aciduria type I (GA-I) via intracerebroventricular delivery of GCDH
Glutaric aciduria type I (GA-I) is an autosomal recessive genetic disorder caused by a deficiency in glutaryl-CoA dehydrogenase (GCDH). Patients who do not receive proper treatment may die from acute encephalopathic crisis.
Lu Guo+12 more
doaj
We determined mRNA expression of the ionotropic glutamate receptors NMDA (NR1, NR2A and NR2B subunits), AMPA (GluR2 subunit) and kainate (GluR6 subunit), as well as of the glutamate transporters GLAST and GLT1 in cerebral cortex and striatum of wild type
Valeska Lizzi Lagranha+9 more
doaj +1 more source
Acute renal proximal tubule alterations during induced metabolic crises in a mouse model of glutaric aciduria type 1 [PDF]
The metabolic disorder glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of the pathologic metabolites glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in blood ...
Braulke, Thomas+7 more
core +1 more source