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  • Mutations
  • Mutations in the following genes cause maple syrup urine disease: BCKDHA (Online Mendelian Inheritance in Man (OMIM) 608348) BCKDHB (Online Mendelian Inheritance in Man (OMIM) 248611) DBT (Online Mendelian Inheritance in Man (OMIM) 248610) DLD (Online Mendelian Inheritance in Man (OMIM) 238331) These four genes produce proteins that work together as the branched-chain alpha-keto acid dehydrogenase complex. (wikipedia.org)
  • Cofactors
  • The succinate dehydrogenase complex showing several cofactors, including flavin , iron-sulfur centers , and heme . (wikipedia.org)
  • Cofactors can be subclassified as either inorganic ions or complex organic molecules called coenzymes , the latter of which is mostly derived from vitamins and other organic essential nutrients in small amounts. (wikipedia.org)
  • ketone
  • The thiazolium ring of TPP is in a zwitterionic form, and the anionic C2 carbon performs a nucleophilic attack on the C2 (ketone) carbonyl of pyruvate. (wikipedia.org)
  • mutation
  • Mutation in any of these genes reduces or eliminates the function of the enzyme complex, preventing the normal breakdown of isoleucine, leucine, and valine. (wikipedia.org)
  • dichloroacetate
  • Apparent K i values of the isoenzymes for the synthetic analogue of pyruvate, dichloroacetate, varied 40-fold, from 0.2 mM for PDK2 to 8 mM for PDK3. (biochemj.org)
  • acetyl group
  • The E1 complex specifically uses the TPP cofactor to cleave the Calpha-C(=O) bond of pyruvate, and then transfer the acetyl group to the TPP coenzyme, thus resulting in an intermediate, hydroxylethyl-Tpp*E1, and producing CO2. (wikipedia.org)
  • serine
  • In this research, we have constructed E. coli strains to produce PHB by engineering the Serine-Deamination (SD) pathway, the Entner-Doudoroff (ED) pathway, and the pyruvate dehydrogenase (PDH) complex. (biomedcentral.com)
  • Significant levels of PHB biosynthesis from different kinds of carbon sources can be achieved by engineering the Serine-Deamination pathway, Entner-Doudoroff pathway and pyruvate dehydrogenase complex in E. coli JM109 harboring the PHB biosynthesis genes from Ralstonia eutropha . (biomedcentral.com)
  • glucose
  • The observed responses to reduced glucose uptake and PEP to pyruvate rate of conversion caused by PTS, PykA and PykF inactivation included flux rerouting in several central metabolism nodes towards anabolic biosynthetic reactions, thus compensating for carbon limitation in these mutant strains. (biomedcentral.com)
  • Metabolism is usually divided into two categories: catabolism, the breaking down of organic matter for example, the breaking down of glucose to pyruvate, by cellular respiration, and anabolism, the building up of components of cells such as proteins and nucleic acids. (wikipedia.org)
  • Crabtree-positive yeasts also have increased glycolytic flow, or increased uptake of glucose and conversion to pyruvate, which compensates for using a portion of the glucose to produce ethanol rather than biomass. (wikipedia.org)
  • Glycolysis (from glycose, an older term for glucose + -lysis degradation) is the metabolic pathway that converts glucose C6H12O6, into pyruvate, CH3COCOO− + H+. (wikipedia.org)
  • For simple fermentations, the metabolism of one molecule of glucose to two molecules of pyruvate has a net yield of two molecules of ATP. (wikipedia.org)
  • kinase
  • A preliminary proteomic survey of rectus abdominus muscle detected a statistically significant increase in adenylate kinase (AK)1, glyceraldehyde-3-phosphate dehydrogenase (GAPDH), and aldolase A in obese/overweight and morbidly obese women relative to lean control subjects. (diabetesjournals.org)
  • This preliminary survey of soluble cytosolic extracts identified adenylate kinase (AK)1, glyceraldehyde-3-phosphate dehydrogenase (GAPDH), and aldolase A as being preferentially expressed in muscle extracts from obese and morbidly obese individuals relative to lean control subjects. (diabetesjournals.org)
  • respiratory chain
  • The origin of aerobic fermentation, or the first step, in Saccharomyces crabtree-positive yeasts likely occurred in the interval between the ability to grow under anaerobic conditions, horizontal transfer of anaerobic DHODase (encoded by URA1 with bacteria), and the loss of respiratory chain Complex I. A more pronounced Crabtree effect, the second step, likely occurred near the time of the WGD event. (wikipedia.org)
  • catalyzes
  • L-lactate dehydrogenases catalyzes the conversion of L-lactate to pyruvate, the last step in anaerobic glycolysis. (wikipedia.org)
  • This complex requires the following 5 coenzymes: Thiamine pyrophosphate Flavin adenine dinucleotide (FAD) Nicotinamide adenine dinucleotide (NAD+) Lipoate Coenzyme A In animal tissue, BCKDC catalyzes an irreversible step in the catabolism of the branched-chain amino acids L-isoleucine, L-valine, and L-leucine, acting on their deaminated derivatives (L-alpha-keto-beta-methylvalerate, alpha-ketoisovalerate, and alpha-ketoisocaproate, respectively). (wikipedia.org)
  • reaction
  • The BCKDC will also oxidize pyruvate, but at such a slow rate this side reaction has very little physiological significance. (wikipedia.org)
  • metabolic
  • Metabolic flux analysis performed on strains lacking the main activities generating pyruvate from PEP revealed the high degree of flexibility to perturbations of the central metabolic network in E. coli . (biomedcentral.com)
  • intermediate
  • This is then converted back to pyruvate (through a malate intermediate), to release the CO2 in the deeper layer of bundle sheath cells for carbon fixation by RuBisCO and the Calvin cycle. (wikipedia.org)
  • molecular
  • X-ray structure of h LDH5 complexes as well as molecular modeling studies contribute to identify and explain the main binding modes of h LDH5 inhibitors reported in literature. (mdpi.com)
  • New techniques for the unbiased ascertainment of complex molecular events in diseased, damaged, and exercise-adapted skeletal muscle include the use of oligonucleotide microarrays and proteomic analysis using mass spectroscopy ( 7 - 10 ). (diabetesjournals.org)
  • activities
  • We find that oxygen consumption, ΔΨ generation, adenine nucleotide translocator-dependent ADP/ATP exchange and both mitochondrial Complex I and IV activities are impaired in CF cells, while both mitochondrial ROS production and membrane lipid peroxidation increase. (springer.com)