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Tanshinone I Attenuates the Effects of a Challenge with H2O2 on the Functions of Tricarboxylic Acid Cycle and Respiratory Chain in SH-SY5Y Cells
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Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria
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Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria
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Manek Rachna, Zhang Yao V., Berthelette Patricia, Hossain Mahmud, Cornell Cathleen S., Gans Joseph, Anarat-Cappillino Gulbenk, Geller Sarah, Jackson Robert, Yu Dan, Singh Kuldeep, Ryan Sue, Bangari Dinesh S., Xu Ethan Y., Kyostio-Moore Sirkka R. M., Scientific Reports
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Modeling the cognitive effects of diet discontinuation in adults with phenylketonuria (PKU) using pegvaliase therapy in PAH-deficient mice
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Winn Shelley R., Dudley Sandra, Scherer Tanja, Rimann Nicole, Th??ny Beat, Boutros Sydney, Krenik Destine, Raber Jacob, Harding Cary O., Molecular Genetics and Metabolism
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Lower plasma cholesterol, LDL-cholesterol and LDL-lipoprotein subclasses in adult phenylketonuria (PKU) patients compared to healthy controls: results of NMR metabolomics investigation
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Cannet Claire, Pilotto Andrea, Rocha J?olio C??sar, Sch?¤fer Hartmut, Spraul Manfred, Berg Daniela, Nawroth Peter, Kasperk Christian, Gramer Gwendolyn, Haas Dorothea, Piel David, K??lker Stefan, Hoffmann Georg, Freisinger Peter, Trefz Friedrich, Orphanet Journal of Rare Diseases
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The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model
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Screening for inherited metabolic diseases using gas chromatography-tandem mass spectrometry (GC-MS/MS) in Sichuan, China
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Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders
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Mutational spectrum of the phenylalanine hydroxylase gene in patients with phenylketonuria in the central region of China
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Shegani Antonio, Kealey Steven, Luzi Federico, Basagni Filippo, Machado Joana do Mar, Ekici Sevban Do??an, Ferocino Alessandra, Gee Antony D., Bongarzone Salvatore, Chemical Reviews
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Consolidated Biochemical Profile of Subacute Stage Traumatic Brain Injury in Early Development
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The Utility of Genomic Testing for Hyperphenylalaninemia
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Tendi Elisabetta Anna, Guarnaccia Maria, Morello Giovanna, Cavallaro Sebastiano, Journal of Clinical Medicine
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Transcription factor-based biosensor for detection of phenylalanine and tyrosine in urine for diagnosis of phenylketonuria
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Evaluation of the Consumption of Fruits and Vegetables by Phenylketonurics in the Metabolic Control of Phenylalanine: An Integrative Review
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Minighin Elaine Carvalho, de Sousa Roberto C??sar Santos, Ramos Ana Luiza Coeli Cruz, Dias Lorena Thais Souza, Labanca Renata Adriana, de Ara?ojo Raquel Linhares Bello, Journal of Medicinal Food
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Ncl1-mediated metabolic rewiring critical during metabolic stress
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Bhat Ajay, Chakraborty Rahul, Adlakha Khushboo, Agam Ganesh, Chakraborty Kausik, Sengupta Shantanu, Life Science Alliance
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Insights from Animal Models on the Pathophysiology of Hyperphenylalaninemia: Role of Mitochondrial Dysfunction, Oxidative Stress and Inflammation
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Wyse Angela T. S., dos Santos Tiago M., Seminotti Bianca, Leipnitz Guilhian, Molecular Neurobiology
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A Retrospective Case Series Analysis of the Relationship Between Phenylalanine: Tyrosine Ratio and Cerebral Glucose Metabolism in Classical Phenylketonuria and Hyperphenylalaninemia
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McGinnity Colm J., Ria?±o Barros Daniela A., Guedj Eric, Girard Nadine, Symeon Christopher, Walker Helen, Barrington Sally F., Summers Mary, Pitkanen Mervi, Rahman Yusof, Frontiers in Neuroscience
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Nutritional and metabolic parameters of children and adolescents with phenylketonuria
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de Almeida Barbara Nara de Freitas, Laufer July Ane, Mezzomo Thais Regina, Shimada Nat??lia Cristina, Furtado Ivy Hulbert Falc?￡o, Dias Marcia Regina Messaggi Gomes, Pereira Rosana Marques, Clinical Nutrition ESPEN
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Of mice and men: Plasma phenylalanine reduction in PKU corrects neurotransmitter pathways in the brain
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The Role of Bacterial-Derived Aromatic Amino Acids Metabolites Relevant in Autism Spectrum Disorders: A Comprehensive Review
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