Results 31 to 40 of about 1,541 (147)

Different Gene Preferences of Maple Syrup Urine Disease in the Aboriginal Tribes of Taiwan

Pediatrics and Neonatology, 2014
Maple syrup urine disease (MSUD) is a rare inborn error of metabolism caused by a deficiency of the branched-chain α-ketoacid dehydrogenase (BCKD) complex. Mutations in any one of the three different genes encoding for the BCKD components, namely, BCKDHA,
Jia-Woei Hou, Tsann-Long Hwang
doaj   +1 more source

Lacticacidaemia due to pyruvate dehydrogenase deficiency, with evidence of protein polymorphism in the α-subunit of the enzyme [PDF]

, 1986
In three infants with neonatal lacticacidaemia, a deficiency in the E 1 (pyruvate dehydrogenase) component of the pyruvate dehydrogenase complex was demonstrated in skin fibroblast cultures. Residual activites of the pyruvate dehydrogenase complex in the
BH Robinson, BH Robinson, DC Devivo, DF Farrel, FJ Ballard, JH Stromme, JP Blass, JP Blass, JP Blass, JP Blass, K Hyland, K Toshimo, KFR Sheu, LJ Reed, LJ Reed, PJ Butterworth, S Sorbi, TE Linn, TW Farmer   +18 more
core   +1 more source

Formation of reactive oxygen species by human and bacterial pyruvate and 2- oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components [PDF]

, 2015
Individual recombinant components of pyruvate and 2-oxoglutarate dehydrogenase multienzyme complexes (PDHc, OGDHc) of human and Escherichia coli (E. coli) origin were expressed and purified from E. coli with optimized protocols.
Adam-Vizi, Adam-Vizi, Adam-Vizi, Ambrus, Ambrus, Ambrus, Ambrus, Ambrus, Andreyev, Attila Ambrus, Azzi, Babady, Balakrishnan, Bando, Beata Torocsik, Berg, Blass, Brautigam, Brautigam, Brown, Bunik, Bunik, Cameron, Chinopoulos, Chinopoulos, Constantinescu, Contreras, Cruts, Diaz-Munoz, Droge, Dumont, Erfle, Fisher-Wellman, Frank, Frank Jordan, Gazaryan, Gibson, Gibson, Gibson, Gibson, Graf, Guest, Gupta, Hamada, Hansford, Harris, Hirashima, Hong, Huennekens, Kiselevsky, Klyachko, Koike, Koike, Korotchkina, Korotchkina, Kumar, Kussmaul, Lai, Lakaschus, Laszlo Tretter, Martinvalet, Massey, Massey, Massey, Mattias Nilsson, Mayo, McCord, McLennan, Mottley, Nakano, Natalia S. Nemeria, Nemeria, Nemeria, Nemeria, Nulton-Persson, Otulakowski, Patel, Perham, Pettit, Popov, Poulsen, Quinlan, Quinonez, Reed, Reed, Reed, Reed, Rosen, Saumweber, Seifert, Shaag, Shany, Sheu, Shi, Shim, Siebels, Song, Starkov, Starkov, Starkov, Tahara, Thekkumkara, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tretter, Tyagi, Vaubel, Vera Adam-Vizi, Vereczki, Wei, Willems, Yan, Yang, Yi, Zundorf   +123 more
core   +1 more source

Cuproptosis: potential new direction in diabetes research and treatment. [PDF]

Front Endocrinol (Lausanne)
Cuproptosis, a recently discovered form of cell death, stems from an overabundance of copper ions infiltrating mitochondria. These ions directly engage lipoylated proteins, prompting their oligomerization and subsequent loss of iron-sulfur clusters. This
Qu J, Wang Y, Wang Q.
europepmc   +3 more sources

Catalysis of transthiolacylation in the active centers of dihydrolipoamide acyltransacetylase components of 2‐oxo acid dehydrogenase complexes

FEBS Open Bio, Volume 8, Issue 6, Page 880-896, June 2018., 2018
Two residues—Asp374 and His375—were identified to be catalytically important at the active center of the E2 component of the Escherichia coli 2‐oxoglutarate dehydrogenase complex. A series of experiments demonstrated that the mechanism of succinyl transfer between the thiol groups of dihydrolipoamide‐E2 and CoA does not involve acid–base catalysis ...
Joydeep Chakraborty, Natalia S. Nemeria, Edgardo Farinas, Frank Jordan   +3 more
wiley   +1 more source

Human Metabolic Enzymes Deficiency: A Genetic Mutation Based Approach

Scientifica, Volume 2016, Issue 1, 2016., 2016
One of the extreme challenges in biology is to ameliorate the understanding of the mechanisms which emphasize metabolic enzyme deficiency (MED) and how these pretend to have influence on human health. However, it has been manifested that MED could be either inherited as inborn error of metabolism (IEM) or acquired, which carries a high risk of ...
Swati Chaturvedi, Ashok K. Singh, Amit K. Keshari, Siddhartha Maity, Srimanta Sarkar, Sudipta Saha, Gary Lopaschuk   +6 more
wiley   +1 more source

Plasmids in the aphid endosymbiont Buchnera aphidicola with the smallest genomes. A puzzling evolutionary story.

, 2006
Buchnera aphidicola, the primary endosymbiont of aphids, has undergone important genomic and biochemical changes as an adaptation to intracellular life.
Gil, Rosario, Latorre, Amparo, Perez-Brocal, Vicente, Sabater-Muñoz, Beatriz, Silva, Francisco J   +4 more
core   +1 more source

Comparative analysis of two genomic regions among four strains of Buchnera aphidicola, primary endosymbiont of aphids.

, 2004
Preliminary analysis of two selected genomic regions of Buchnera aphidicola BCc, the primary endosymbiont of the cedar aphid Cinara cedri, has revealed a number of interesting features when compared with the corresponding homologous regions of the three ...
Gil, Rosario, Latorre, Amparo, Moya, Andrés, Pérez-Brocal, Vicente   +3 more
core   +1 more source

LC-MS proteomics analysis of the iInsulin/IGF-1-deficient Caenorhabditis elegans daf-2(e1370) mutant reveals extensive restructuring of intermediary metabolism [PDF]

, 2014
The insulin/IGF-1 receptor is a major known determinant of dauer formation, stress resistance, longevity, and metabolism in Caenorhabditis elegans. In the past, whole-genome transcript profiling was used extensively to study differential gene expression ...
Braeckman, Bart, Brewer, Heather M, Camp, David G, II, Depuydt, Geert, Petyuk, Vladislav A, Smith, Richard D, Smolders, Arne, Xie, Fang   +7 more
core   +2 more sources

Structure of the cubic core and the lipoamide dehydrogenase component of the pyruvate dehydrogenase complex. [PDF]

, 1992
In this thesis, the crystal structures of the E3 component and of the catalytic domain of E2p are presented. These are the first atomic structures of the cubic core and of the E3 component of the pyruvate dehydrogenase and related multienzyme complexes ...
Mattevi, Andrea
core   +3 more sources