Results 211 to 220 of about 42,696 (258)

Advancements in metabolic engineering: unlocking the potential of key organic acids for sustainable industrial applications. [PDF]

Front Bioeng Biotechnol
Wang T, Xue H, Liu H, Yuan H, Huang D, Jiang Y.   +5 more
europepmc   +1 more source

Melatonin Improves Lipid Homeostasis, Mitochondrial Biogenesis, and Antioxidant Defenses in the Liver of Prediabetic Rats. [PDF]

Int J Mol Sci
de Souza MC, Agneis MLG, das Neves KA, de Almeida MR, Feltran GDS, Souza Cruz EM, Schoffen JPF, Chuffa LGA, Seiva FRF.   +8 more
europepmc   +1 more source

Type 3 diabetes and metabolic reprogramming of brain neurons: causes and therapeutic strategies. [PDF]

Mol Med
Meng X, Zhang H, Zhao Z, Li S, Zhang X, Guo R, Liu H, Yuan Y, Li W, Song Q, Liu J.   +10 more
europepmc   +1 more source

Time-resolved mitochondrial screen identifies regulatory components of oxidative metabolism. [PDF]

EMBO Rep
Zamora-Dorta M, Laine-Menéndez S, Abia D, González-García P, López LC, Fernández-Montes P, Calvo E, Vázquez J, Enríquez JA, Balsa E.   +9 more
europepmc   +1 more source

LIPT1 deficiency presenting as early infantile epileptic encephalopathy, Leigh disease, and secondary pyruvate dehydrogenase complex deficiency

American Journal of Medical Genetics Part A, 2018
Lipoic acid is an essential cofactor for the mitochondrial 2‐ketoacid dehydrogenase complexes and the glycine cleavage system. Lipoyltransferase 1 catalyzes the covalent attachment of lipoate to these enzyme systems. Pathogenic variants in LIPT1 gene have recently been described in four patients from three families, commonly presenting with severe ...
Robert C. Stowe, Qin Sun, Sarah H. Elsea, Fernando Scaglia   +3 more
semanticscholar   +5 more sources

Pyruvate Dehydrogenase Complex Deficiency as a Cause of Subacute Necrotizing Encephalopathy (Leigh Disease)

Pediatrics, 1987
Leigh disease is a disorder with great clinical variability and for which diverse biochemical causes have been proposed. Clarification requires rigorous correlation of biochemical abnormalities with strict morphologic diagnosis; such an unambiguous ...
Hans A. Kretzschmar, Stephen J. DeArmond, Thomas Koch, Mulchand S. Patel, Christopher J. L. Newth, Kathleen A. Schmidt, Seymour Packman   +6 more
semanticscholar   +4 more sources

Deficiency of pyruvate dehydrogenase complex (PDHC) in Leigh's disease fibroblasts

Neurology, 1989
Several groups have reported abnormalities of the pyruvate dehydrogenase complex (PDHC) in cultured cells or other tissues from patients with Leigh's disease (subacute necrotizing encephalomyelopathy). We therefore undertook studies to elucidate the molecular basis of the defect of PDHC in cultured skin fibroblasts from two patients with Leigh's ...
Lois M. Hinman, K‐F. REX SHEU, Andrew C. Baker, Y. T. Kim, John P. Blass   +4 more
semanticscholar   +5 more sources