Results 151 to 160 of about 5,746 (195)

Role of novel protein acylation modifications in sepsis. [PDF]

Front Immunol
Wang J, He A, Song L, Jiang W, Xu L, Zheng R, Yu J.   +6 more
europepmc   +1 more source

Mapping Disorders with Neurological Features Through Mitochondrial Impairment Pathways: Insights from Genetic Evidence. [PDF]

Curr Issues Mol Biol
Makridou A, Sintou E, Chatzianagnosti S, Dermitzakis I, Gargani S, Manthou ME, Theotokis P.   +6 more
europepmc   +1 more source

Early Metabolic Profile in Neonates with Maternal Intrahepatic Cholestasis of Pregnancy. [PDF]

Children (Basel)
Guner Yilmaz B, Abali S, Moniri A, Kilinckaya U, Altinbas E, Ay B, Karakose B, Sahinoglu Y, Sahinoglu M, Yilmaz B, Serteser M, Korkmaz A, Pata O, Beken S.   +13 more
europepmc   +1 more source

Characterization of C5 Acylcarnitines and Related Dicarboxylic Acylcarnitines in Saudi Newborns: Screening, Confirmation, and Cutoff Variation. [PDF]

Int J Neonatal Screen
Bokhari HA, Mujamammi AH, Bader HA, Alamri HJ, Alharbi KK.   +4 more
europepmc   +1 more source

Structure Elucidation for MALDI Mass Spectrometry Imaging Using Infrared Ion Spectroscopy. [PDF]

Anal Chem
Schuurman JL, van Tetering L, Houthuijs KJ, Kooijman P, Gailus-Durner V, Leuchtenberger S, Fuchs H, Hrabe de Angelis M, Engelke UFH, Lefeber DJ, van Karnebeek CDM, Wevers RA, Grgic A, Balluf B, Vandenbosch M, Vreeken R, Heeren RMA, Berden G, Oomens J, Martens J.   +19 more
europepmc   +1 more source

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Kinetic Mechanism of Glutaryl-CoA Dehydrogenase

Biochemistry, 2006
Glutaryl-CoA dehydrogenase (GCD) is a homotetrameric enzyme containing one noncovalently bound FAD per monomer that oxidatively decarboxylates glutaryl-CoA to crotonyl-CoA and CO2. GCD belongs to the family of acyl-CoA dehydrogenases that are evolutionarily conserved in their sequence, structure, and function.
K Sudhindra, Rao, Mark, Albro, Timothy M, Dwyer, Frank E, Frerman   +3 more
openaire   +2 more sources

Riboflavin-responsive glutaryl CoA dehydrogenase deficiency

Molecular Genetics and Metabolism, 2006
We report here riboflavin responsiveness in a patient with glutaryl CoA dehydrogenase (GCDH) deficiency, compound heterozygous for the S139L and P248L mutations and with 20% residual GCDH enzyme activity in vitro. Our results suggest the mitochondrial GCDH homotetramer remains intact with one of these mutations associated with the binding site of the ...
Ronald A, Chalmers, Murray D, Bain, Johannes, Zschocke   +2 more
openaire   +2 more sources

Mechanism of action of glutaryl-CoA and butyryl-CoA dehydrogenases. Purification of glutaryl-CoA dehydrogenase

Biochemistry, 1981
Glutaryl-CoA dehydrogenase, a flavoprotein, catalyzes the reaction -OOCCH3CH2--CH2COSR (FAD leads to FADH2) leads to CH3CH = CHCOSR + CO2 (SR = CoA or pantetheine). With the isolated enzyme, a dye serves as the final electron acceptor. The enzyme from Pseudomonas fluorescens (ATCC 11250) has been purified to homogeneity.
B, Gomes, G, Fendrich, R H, Abeles
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Emergency treatment in glutaryl‐CoA dehydrogenase deficiency

Journal of Inherited Metabolic Disease, 2004
AbstractSummary: The history of glutaryl‐CoA dehydrogenase deficiency is determined by acute encephalopathic crises that are precipitated by common febrile diseases, vaccinations or surgical interventions during infancy and early childhood. Such crises result in an irreversible destruction of the basal ganglia (in particular of the putamina), and ...
S, Kölker, C R, Greenberg, M, Lindner, E, Müller, E R, Naughten, G F, Hoffmann   +5 more
openaire   +2 more sources