414 results on '"Okun, Jürgen G"'
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102. Fasting‐induced liver GADD 45β restrains hepatic fatty acid uptake and improves metabolic health
103. Co-expression of phenylalanine hydroxylase variants and effects of interallelic complementation on in vitro enzyme activity and genotype-phenotype correlation
104. Significant prevalence of sickle cell disease in Southwest Germany: results from a birth cohort study indicate the necessity for newborn screening
105. High incidence of maternal vitamin B12 deficiency detected by newborn screening: first results from a study for the evaluation of 26 additional target disorders for the German newborn screening panel.
106. Increased storage and secretion of phosphatidylcholines by senescent human peritoneal mesothelial cells
107. Molecular regulation of urea cycle function by the liver glucocorticoid receptor
108. Significance of the Extent of Intestinal Resection on the Outcome of a Short-bowel Syndrome in a Porcine Model
109. Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I
110. Neugeborenenscreening auf Mukoviszidose in Deutschland: Vergleich des neuen Screening-Protokolls mit einem Alternativprotokoll.
111. Dietary tryptophan is required for CNS infiltration of encephalitogenic T cells
112. Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I
113. The β-hydroxybutyrate receptor HCA2 activates a neuroprotective subset of macrophages
114. Diagnosis and therapeutic monitoring of inborn errors of creatine metabolism and transport using liquid chromatography–tandem mass spectrometry in urine, plasma and CSF
115. BCAT1 promotes cell proliferation through amino acid catabolism in gliomas carrying wild-type IDH1
116. Significant prevalence of sickle cell disease in Southwest Germany: results from a birth cohort study indicate the necessity for newborn screening.
117. Significance of the Extent of Intestinal Resection on the Outcome of a Short-bowel Syndrome in a Porcine Model.
118. DHTKD1 Mutations Cause 2-Aminoadipic and 2-Oxoadipic Aciduria
119. Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b−/− mice as a model for Wilson disease
120. Detection of 2-Hydroxyglutarate in Formalin-Fixed Paraffin-Embedded Glioma Specimens by Gas Chromatography/Mass Spectrometry
121. Ubiquinol-induced gene expression signatures are translated into altered parameters of erythropoiesis and reduced low density lipoprotein cholesterol levels in humans
122. The reduced form of coenzyme Q10 mediates distinct effects on cholesterol metabolism at the transcriptional and metabolite level in SAMP1 mice
123. Therapeutic modulation of cerebral l-lysine metabolism in a mouse model for glutaric aciduria type I
124. Glutaric aciduria type I and methylmalonic aciduria: Simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood–brain barrier and the choroid plexus
125. Mitochondrial Reactive Oxygen Species Control T Cell Activation by Regulating IL-2 and IL-4 Expression: Mechanism of Ciprofloxacin-Mediated Immunosuppression
126. Xanthohumol‐induced transient superoxide anion radical formation triggers cancer cells into apoptosis via a mitochondria‐mediated mechanism
127. Deorphanization of GPR109B as a Receptor for the β-Oxidation Intermediate 3-OH-octanoic Acid and Its Role in the Regulation of Lipolysis
128. Nitrous oxide promotes hyperhomocysteinemia in levodopa treated rats
129. Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins
130. Comprehensive Detection of Disorders of Purine and Pyrimidine Metabolism by HPLC with Electrospray Ionization Tandem Mass Spectrometry
131. Phenylalanine Reduces Synaptic Density in Mixed Cortical Cultures from Mice
132. Quantitative Acylcarnitine Profiling in Peripheral Blood Mononuclear Cells Using In Vitro Loading With Palmitic and 2-Oxoadipic Acids: Biochemical Confirmation of Fatty Acid Oxidation and Organic Acid Disorders
133. Bioenergetics in Glutaryl-Coenzyme A Dehydrogenase Deficiency
134. Optimized Spectrophotometric Assay for the Completely Activated Pyruvate Dehydrogenase Complex in Fibroblasts
135. Synthesis and metabolism of leukotrienes in γ-glutamyl transpeptidase deficiency
136. Simultaneous analysis of coenzyme Q10 in plasma, erythrocytes and platelets: comparison of the antioxidant level in blood cells and their environment in healthy children and after oral supplementation in adults
137. Pathomechanisms of neurodegeneration in glutaryl‐CoA dehydrogenase deficiency
138. Methylmalonic Acid, a Biochemical Hallmark of Methylmalonic Acidurias but No Inhibitor of Mitochondrial Respiratory Chain
139. Coenzyme Q10 in plasma and erythrocytes: comparison of antioxidant levels in healthy probands after oral supplementation and in patients suffering from sickle cell anemia
140. A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency
141. Ca2+ and Na+ Dependence of 3-Hydroxyglutarate-Induced Excitotoxicity in Primary Neuronal Cultures from Chick Embryo Telencephalons
142. NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration ind-2-hydroxyglutaric aciduria
143. Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons
144. Neurodegeneration in Methylmalonic Aciduria Involves Inhibition of Complex II and the Tricarboxylic Acid Cycle, and Synergistically Acting Excitotoxicity
145. 3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: A hypothesis
146. Three Molecules of Ubiquinone Bind Specifically to Mitochondrial Cytochrome bc 1Complex
147. Binding of detergents and inhibitors to bovine complex I - a novel purification procedure for bovine complex I retaining full inhibitor sensitivity
148. Properties of the Common Inhibitor Binding Domain in Mitochondrial NADH-Dehydrogenase (Complex I)
149. Three Classes of Inhibitors Share a Common Binding Domain in Mitochondrial Complex I (NADH:Ubiquinone Oxidoreductase)
150. Role of Deprotonation Events in Ubihydroquinone:Cytochrome c Oxidoreductase from Bovine Heart and Yeast Mitochondria
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