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Academic Journal

Imaging brain glucose metabolism in vivo reveals propionate as a major anaplerotic substrate in pyruvate dehydrogenase deficiency.

  • Authors : Marin-Valencia I; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA; Laboratory of Developmental Neurobiology, The Rockefeller University, New York, NY, USA. Electronic address: .

Subjects: Pyruvate Dehydrogenase Complex Deficiency Disease*/Pyruvate Dehydrogenase Complex Deficiency Disease*/Pyruvate Dehydrogenase Complex Deficiency Disease*/metabolism ; Brain*/Brain*/Brain*/metabolism ; Brain*/Brain*/Brain*/diagnostic imaging

  • Source: Cell metabolism [Cell Metab] 2024 Jun 04; Vol. 36 (6), pp. 1394-1410.e12.Publisher: Cell Press Country of Publication: United States NLM ID: 101233170 Publication Model: Print Cited Medium: Internet ISSN: 1932-7420

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Academic Journal

Magnetic resonance spectroscopic investigation of mitochondrial fuel metabolism and energetics in cultured human fibroblasts: effects of pyruvate dehydrogenase complex deficiency and dichloroacetate.

  • Authors : Simpson NE; Department of Medicine, Division of Endocrinology and Metabolism, University of Florida, Gainesville, FL 32610, USA. ; Han Z

Subjects: Glucose/Glucose/Glucose/*metabolism ; Magnetic Resonance Spectroscopy/Magnetic Resonance Spectroscopy/Magnetic Resonance Spectroscopy/*methods ; Mitochondria/Mitochondria/Mitochondria/*metabolism

  • Source: Molecular genetics and metabolism [Mol Genet Metab] 2006 Sep-Oct; Vol. 89 (1-2), pp. 97-105. Date of Electronic Publication: 2006 Jun 12.Publisher: Academic Press Country of Publication: United States NLM ID: 9805456 Publication Model: Print-Electronic Cited Medium: Print ISSN:

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Determination of [U-13C]glucose turnover into various metabolite pools for the differential diagnosis of lactic acidemias.

  • Authors : Kassel DB; Department of Chemistry, Michigan State University, East Lansing 48824.; Glerum M

Subjects: Cytochrome-c Oxidase Deficiency*; Glucose/Glucose/Glucose/*metabolism ; Lactates/Lactates/Lactates/*blood

  • Source: Analytical biochemistry [Anal Biochem] 1989 Feb 01; Vol. 176 (2), pp. 382-9.Publisher: Elsevier Country of Publication: United States NLM ID: 0370535 Publication Model: Print Cited Medium: Print ISSN: 0003-2697 (Print)

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Academic Journal

Energy substrate metabolism in pyruvate dehydrogenase complex deficiency.

Subjects: Energy Metabolism* ; Lipolysis*; Glucose/Glucose/Glucose/*metabolism

  • Source: Journal of pediatric endocrinology & metabolism : JPEM [J Pediatr Endocrinol Metab] 2014 Nov; Vol. 27 (11-12), pp. 1059-64.Publisher: Walter de Gruyter Country of Publication: Germany NLM ID: 9508900 Publication Model: Print Cited Medium: Internet ISSN: 2191-0251

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  • 1-4 of  4 results for ""Pyruvate Dehydrogenase Complex Deficiency Disease""