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Material Type: Artigo
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Obesity-related elevations in plasma leucine are associated with alterations in enzymes involved in branched-chain amino acid metabolismShe, Pengxiang ; Van Horn, Cynthia ; Reid, Tanya ; Hutson, Susan M ; Cooney, Robert N ; Lynch, Christopher JAmerican journal of physiology: endocrinology and metabolism, 2007-12, Vol.293 (6), p.E1552-E1563 [Periódico revisado por pares]United States: American Physiological SocietyTexto completo disponível |
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Material Type: Artigo
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Two Novel Mutations in the BCKDK (Branched-Chain Keto-Acid Dehydrogenase Kinase) Gene Are Responsible for a Neurobehavioral Deficit in Two Pediatric Unrelated PatientsGarcía-Cazorla, Angels ; Oyarzabal, Alfonso ; Fort, Joana ; Robles, Concepción ; Castejón, Esperanza ; Ruiz-Sala, Pedro ; Bodoy, Susanna ; Merinero, Begoña ; Lopez-Sala, Anna ; Dopazo, Joaquín ; Nunes, Virginia ; Ugarte, Magdalena ; Artuch, Rafael ; Palacín, Manuel ; Rodríguez-Pombo, Pilar ; Alcaide, Patricia ; Navarrete, Rosa ; Sanz, Paloma ; Font-Llitjós, Mariona ; Vilaseca, Ma Antonia ; Ormaizabal, Aida ; Pristoupilova, Anna ; Agulló, Sergi BeltranHuman mutation, 2014-04, Vol.35 (4), p.470-477 [Periódico revisado por pares]United States: Blackwell Publishing LtdTexto completo disponível |
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Material Type: Artigo
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Living donor liver transplantation from a heterozygous parent for classical maple syrup urine diseaseKadohisa, Masashi ; Matsumoto, Shirou ; Sawada, Hirotake ; Honda, Masaki ; Murokawa, Takahiro ; Hayashida, Shintaro ; Ohya, Yuki ; Lee, Kwang‐Jong ; Yamamoto, Hidekazu ; Mitsubuchi, Hiroshi ; Endo, Fumio ; Inomata, YukihiroPediatric transplantation, 2015-05, Vol.19 (3), p.E66-E69 [Periódico revisado por pares]DenmarkTexto completo disponível |
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Material Type: Artigo
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A molecular model of human branched-chain amino acid metabolismSURYAWAN, A ; HAWES, J. W ; HARRIS, R. A ; SHIMOMURA, Y ; JENKINS, A. E ; HUTSON, S. MThe American journal of clinical nutrition, 1998-07, Vol.68 (1), p.72-81 [Periódico revisado por pares]Bethesda, MD: American Society for Clinical NutritionTexto completo disponível |
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5 |
Material Type: Artigo
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Successful treatment of severe MSUD in Bckdhb−/− mice with neonatal AAV gene therapyPontoizeau, Clément ; Gaborit, Clovis ; Tual, Nolan ; Simon‐Sola, Marcelo ; Rotaru, Irina ; Benoist, Marion ; Colella, Pasqualina ; Lamazière, Antonin ; Brassier, Anaïs ; Arnoux, Jean‐Baptiste ; Rötig, Agnès ; Ottolenghi, Chris ; Lonlay, Pascale ; Mingozzi, Federico ; Cavazzana, Marina ; Schiff, ManuelJournal of inherited metabolic disease, 2024-01, Vol.47 (1), p.41-49 [Periódico revisado por pares]Hoboken, USA: John Wiley & Sons, IncTexto completo disponível |
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Material Type: Artigo
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AICAR stimulates mitochondrial biogenesis and BCAA catabolic enzyme expression in C2C12 myotubesHinkle, Jason S. ; Rivera, Caroline N. ; Vaughan, Roger A.Biochimie, 2022-04, Vol.195, p.77-85 [Periódico revisado por pares]France: Elsevier B.VTexto completo disponível |
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Material Type: Artigo
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Exercise promotes BCAA catabolism: effects of BCAA supplementation on skeletal muscle during exerciseShimomura, Y ; Murakami, T ; Nakai, N ; Nagasaki, M ; Harris, R.AThe Journal of nutrition, 2004-06, Vol.134 (6S), p.1583S-1587S [Periódico revisado por pares]Bethesda, MD: American Society for Nutritional SciencesTexto completo disponível |
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Material Type: Artigo
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Conformational stability and thermodynamic characterization of the lipoic acid bearing domain of human mitochondrial branched chain α‐ketoacid dehydrogenaseNaik, Mandar T. ; Huang, Tai‐HuangProtein science, 2004-09, Vol.13 (9), p.2483-2492 [Periódico revisado por pares]Bristol: Cold Spring Harbor Laboratory PressTexto completo disponível |
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9 |
Material Type: Artigo
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Catabolism of Branched-Chain Amino Acids in Heart Failure: Insights from Genetic ModelsSun, Haipeng ; Lu, Gang ; Ren, Shuxun ; Chen, Jaunian ; Wang, YibinPediatric cardiology, 2011-03, Vol.32 (3), p.305-310 [Periódico revisado por pares]New York: Springer-VerlagTexto completo disponível |
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10 |
Material Type: Artigo
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Maple syrup urine disease: Further evidence that newborn screening may fail to identify variant formsPuckett, R.L. ; Lorey, F. ; Rinaldo, P. ; Lipson, M.H. ; Matern, D. ; Sowa, M.E. ; Levine, S. ; Chang, R. ; Wang, R.Y. ; Abdenur, J.E.Molecular genetics and metabolism, 2010-06, Vol.100 (2), p.136-142 [Periódico revisado por pares]United States: Elsevier IncTexto completo disponível |