introduction: glutaric academia type i is a metabolic disorder that is caused due to deficiency of glutaryl-coa dehydrogenase. macrocephaly is a common sign in ga1, although many infants usually appear healthy at birth.   case report a 5.5 year old boy with ga1was admitted to nicu. chief compliance of patient for hospitalization was pneumonia and sepsis and he was intubated and mechanically ven...

Statins (3-hydroxy-3-methyl glutaryl coenzyme A [HMG-CoA] reductase inhibitors) are the most commonly used lipid-lowering drugs. Their main lipid-lowering effect is achieved by an increase in the expression of low-density lipoprotein cholesterol receptors associated with inhibition of cholesterol synthesis through inhibition of HMG-CoA reductase - the first and rate-limiting step in cholesterol...

The aim of this study was to evaluate hypocholesterolemic potential phytoconstituents ethanolic seed extract cumin (Cuminum cyminum L.) by assessments interaction capabilities with 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMG-CoA) through in vivo and silico along screening the test extract. were identified Gas chromatography-mass spectrometry (GC-MS)/MS examinations. hypercholesterolem...

In a previous study, the essential role of 3-sulfinopropionyl coenzyme A (3SP-CoA) desulfinase acyl-CoA dehydrogenase (Acd) in Advenella mimigardefordensis strain DPN7(T) (AcdDPN7) during degradation of 3,3'-dithiodipropionic acid (DTDP) was elucidated. DTDP is a sulfur-containing precursor substrate for biosynthesis of polythioesters (PTEs). AcdDPN7 showed high amino acid sequence similarity t...

Glutaric aciduria type 1 (GA1) is an inherited neurometabolic disorder caused by mutations in the GCDH gene encoding glutaryl-CoA dehydrogenase (GCDH), which forms homo- and heteromeric complexes in the mitochondrial matrix. GA1 patients are prone to the development of encephalopathic crises which lead to an irreversible disabling dystonic movement disorder. The clinical and biochemical manifes...

Glutaric aciduria type 1 (GA1), resulting from the genetic deficiency of glutaryl-CoA dehydrogenase (GDH), is a relatively common cause of acute metabolic brain damage in infants. Encephalopathic crises may be prevented by carnitine supplementation and diet, but diagnosis can be difficult as some patients do not show the typical excretion of large amounts of glutaric and 3-hydroxyglutaric acids...

A complete pathway for Azorhizobium caulinodans nicotinate catabolism has been determined from mutant phenotype analyses, isolation of metabolic intermediates, and structural studies. Nicotinate serves as a respiratory electron donor to O2 via a membrane-bound hydroxylase and a specific c-type cytochrome oxidase. The resulting oxidized product, 6-hydroxynicotinate, is next reduced to 1,4,5,6-te...

CYP4A6/P450 IV rabbit AACT AGGGCA A AGTTGA [1] CYP4A1/P450 IV rat AACT AGGGTA A AGTTCA [2] L-fatty acid binding protein rat ATAT AGGCCA T AGGTCA* [3] 3-hydroxy-3-methyl-glutaryl-CoA-synthase rat AACT GGGCCA A AGGTCT* [4] Enoyl-CoA-hydratase rat ATGT AGGTAA T AGTTCA* [1] Malic enzyme rat TTCT GGGTCA A AGTTGA [5] Phosphoenolpyruvate carboxikinase rat AACT GGGATA A AGGTCT [6] Phosphoenolpyruvate c...

Introduction: Glutaric academia type I is a metabolic disorder that is caused due to deficiency of glutaryl-CoA dehydrogenase. Macrocephaly is a common sign in GA1, although many infants usually appear healthy at birth.   Case Report A 5.5 year old boy with GA1was admitted to NICU. Chief compliance of patient for hospitalization was pneumonia and sepsis and he was intubated and mechanically ven...