Acid dehydrogenase complex. Medical search. Frequent questions

This enzyme complex is composed of three catalytic components: alpha-ketoacid dehydrogenase (also referred to as the E1 component), dihydrolipoyl transacylase (E2 component), and dihydrolipoamide dehydrogenase (E3 component). (wikipedia.org)
Dihydrolipoamide dehydrogenase deficiency is a severe condition that can affect several body systems. (medlineplus.gov)
A common feature of dihydrolipoamide dehydrogenase deficiency is a potentially life-threatening buildup of lactic acid in tissues (lactic acidosis), which can cause nausea, vomiting, severe breathing problems, and an abnormal heartbeat. (medlineplus.gov)
Liver problems can also occur in dihydrolipoamide dehydrogenase deficiency, ranging from an enlarged liver (hepatomegaly) to life-threatening liver failure. (medlineplus.gov)
Typically, the signs and symptoms of dihydrolipoamide dehydrogenase deficiency occur in episodes that may be triggered by fever, injury, or other stresses on the body. (medlineplus.gov)
Dihydrolipoamide dehydrogenase deficiency occurs in an estimated 1 in 35,000 to 48,000 individuals of Ashkenazi Jewish descent. (medlineplus.gov)
In other populations, the prevalence of dihydrolipoamide dehydrogenase deficiency is unknown, but the condition is likely rare. (medlineplus.gov)
Mutations in the DLD gene cause dihydrolipoamide dehydrogenase deficiency. (medlineplus.gov)
This gene provides instructions for making an enzyme called dihydrolipoamide dehydrogenase (DLD). (medlineplus.gov)
The brain is especially affected by the buildup of molecules and the lack of cellular energy, resulting in the neurological problems associated with dihydrolipoamide dehydrogenase deficiency. (medlineplus.gov)
The cycle of reaction is completed when reduced lipoamide is reoxidised by the flavoprotein, dihydrolipoamide dehydrogenase (E 3 ). (bmj.com)
The L protein, also named dihydrolipoamide dehydrogenase, is also a component of the pyruvate dehydrogenase complex, the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. (thermofisher.com)

The branched-chain α-ketoacid dehydrogenase complex (BCKDC or BCKDH complex) is a multi-subunit complex of enzymes that is found on the mitochondrial inner membrane. (wikipedia.org)
BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family comprising pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, key enzymes that function in the Krebs cycle. (wikipedia.org)
DLD is one component of three different groups of enzymes that work together (enzyme complexes): branched-chain alpha-keto acid dehydrogenase (BCKD), pyruvate dehydrogenase (PDH), and alpha (α)-ketoglutarate dehydrogenase (αKGDH). (medlineplus.gov)
Most identifiable cases involve inherited or spontaneous mutations in the pyruvate dehydrogenase complex (PDC) or in one or more enzymes of the respiratory chain. (bmj.com)
2 4 A few cases have been reported that involve deficiencies in enzymes of the tricarboxylic acid cycle, such as fumarase, or of gluconeogenesis, such as pyruvate carboxylase (PC) or phosphoenolpyruvate carboxykinase (PEPCK). (bmj.com)
Such enzymes include the pyruvate dehydrogenase complex that synthesises the acetyl-CoA needed for the first reaction of the TCA cycle. (citizendium.org)
Also the enzymes citrate synthase, isocitrate dehydrogenase and alpha-ketoglutarate dehydrogenase, that regulate the first three steps of the TCA cycle, are inhibited by high concentrations of ATP. (citizendium.org)
This includes both the entry point enzymes pyruvate dehydrogenase and citrate synthase. (citizendium.org)
In protein catabolism , proteins are broken down by protease enzymes into their constituent amino acids. (citizendium.org)
Amino acid synthesis in yeast cells occurs through metabolic pathways, and enzymes in the synthesis pathways are encoded by different genes. (onlinemathlearning.com)
The BCKD enzyme complex, which is associated with the inner mitochondrial membrane, has 3 different catalytic components (ie, E1, E2, E3) and 2 associated regulatory enzymes (ie, BCKD phosphatase, BCKD kinase). (medscape.com)
Figure shows the ping-pong mechanism for production of lactobionic acid by oxidation of lactose using two enzymes (CDH and Laccase) and an intermediary redox mediator (ABTS). (aiche.org)
Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. (medlineplus.gov)
The complex mainly consists of three enzymes, which are respectively called E1, E2 and E3 components of PDH according to the order in which they participate in the reactions. (biomedcentral.com)
The E 3 subunit is shared with two other enzymes: 2-oxoglutarate dehydrogenase (a citric acid cycle enzyme) and 2-oxo acid dehydrogenase (a enzyme required for amino acid degradation) [ Pyruvate Dehydrogenase Evolution ]. (blogspot.com)
By this way in arsenic poisoning enzymes of the pyruvate dehydrogenase complex and alpha-keto glutarate complex are inhibited by arsenite. (biotechfront.com)
Naturally-occurring autoantibodies to a family of mitochondrial enzymes, the 2-oxoacid dehydrogenase complexes (2-OADC), characterize the human liver disease primary biliary cirrhosis. (edu.au)
The immunogens included the intact pyruvate dehydrogenase complex (PDC) from bovine heart, human recombinant PDC-E2, and short synthetic peptides representing the immunodominant lipoic acid binding sequences of the 2-OADC enzymes. (edu.au)
Some of the enzymes which generate aromatic radicals that break down most prominent bacterial strains found were isolated and tax- the complex linkages present in lignin to compounds of lower onomically identified using 16S ribosomal RNA (rRNA) se- molecular weight. (lu.se)

citric acid cycle]], also known as the tricarboxylic acid cycle (TCA cycle) or the Krebs cycle, (or rarely, the Szent-Gyorgyi-Krebs cycle) is a series of enzyme-catalysed chemical reactions of central importance in all living cells that use oxygen as part of cellular respiration. (wikipathways.org)
In eukaryotes, the citric acid cycle occurs in the matrix of the mitochondrion. (wikipathways.org)
Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme, notable for its role in the synthesis and oxidization of fatty acids, and the oxidation of pyruvate in the citric acid cycle. (dadamo.com)
Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production. (dadamo.com)
Normally, acetyl-CoA from fatty acid metabolism feeds into the citric acid cycle, contributing to the cell's energy supply. (dadamo.com)
[1] In aerobic organisms , the citric acid cycle is involved in the chemical conversion of carbohydrates , fats and proteins into carbon dioxide and water to generate a form of usable energy. (citizendium.org)
The citric acid cycle also provides precursors for many compounds such as certain amino acids , and some of its reactions are therefore important even in cells performing fermentation . (citizendium.org)
The citric acid cycle is the second step in carbohydrate catabolism (the breakdown of sugars). (citizendium.org)
It is converted into acetyl-CoA and enters the citric acid cycle. (citizendium.org)
These amino acids are brought into the cells and can be a source of energy by being funnelled into the citric acid cycle. (citizendium.org)
In many tissues, especially heart tissue, fatty acids are broken down through a process known as beta oxidation which results in acetyl-CoA which can be used in the citric acid cycle. (citizendium.org)
The citric acid cycle is always followed by oxidative phosphorylation . (citizendium.org)
The citric acid cycle itself does not use oxygen, but oxidative phosphorylation does. (citizendium.org)
The pyruvate dehydrogenase complex (PDC) catalyzes the conversion of pyruvate to acetyl-CoA, a substrate for the Krebs (citric acid) cycle. (onlinemathlearning.com)
This is the link reaction between glycolysis and the citric acid cycle . (biotechfront.com)
Succinate:quinone oxidoreductase (SQR) is both a Krebs´ citric acid cycle enzyme and a component (Complex II) of the respiratory chain in aerobic cells. (lu.se)

The E3 component is associated with 2 additional alpha-ketoacid dehydrogenase complexes, namely pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase. (medscape.com)

Reducing equivalents (NADH, FADH 2 ) are generated by reactions catalysed by the PDC and the tricarboxylic acid cycle and donate electrons (e - ) that enter the respiratory chain at NADH ubiquinone oxidoreductase (complex I) or at succinate ubiquinone oxidoreductase (complex II). (bmj.com)
Through a complex reaction, pyruvate is decarboxylated and turned into acetaldehyde, then attached to coenzyme A while NAD+ is subsequently reduced to NADH and H+. (dadamo.com)
PDH kinase which are responsible for the inactive PDH is promoted by ATP, NADH and acetyl coenzyme A. while PDH complex is inhibited by NAD+, coenzyme A and pyruvate. (biotechfront.com)

On the role of the mitochondrial 2-oxoglutarate dehydrogenase complex in amino acid metabolism. (mpg.de)
This severe condition is characterized by impairment of more than one mitochondrial function, such as reduced activity of complex I, II, or III, pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, or the glycine cleavage system. (medlineplus.gov)
Reduced activity of complex I, II, or III, pyruvate dehydrogenase, or alpha-ketoglutarate dehydrogenase leads to potentially fatal lactic acidosis, encephalopathy, and other signs and symptoms of multiple mitochondrial dysfunctions syndrome. (medlineplus.gov)
PDH complex is present on the inner mitochondrial membrane . (biotechfront.com)
Mature frataxin (81-210) is a critically important protein because it facilitates the assembly of mitochondrial iron-sulfur cluster protein complexes such as aconitase, lipoate synthase, and succinate dehydrogenases. (nih.gov)

This modification is also critical to the function of the glycine cleavage system, a set of proteins that breaks down a protein building block (amino acid) called glycine when levels become too high. (medlineplus.gov)

This enzyme complex catalyzes the oxidative decarboxylation of branched, short-chain alpha-ketoacids. (wikipedia.org)
The mechanism of enzymatic catalysis by the BCKDC draws largely upon the elaborate structure of this large enzyme complex. (wikipedia.org)
The inner-core domain is necessary to form the oligomeric core of the enzyme complex and catalyzes the acyltransferase reaction (shown in the "Mechanism" section below). (wikipedia.org)
The BCKD enzyme complex is involved in the breakdown of three protein building blocks (amino acids) commonly found in protein-rich foods: leucine, isoleucine, and valine. (medlineplus.gov)
It is catalyzed by an enzyme-complex called pyruvate dehydrogenase. (dadamo.com)
[ 7 ] The branched-chain alpha-keto acid dehydrogenase (BCKD) enzyme complex was purified and characterized in 1978. (medscape.com)
Maple syrup urine disease is caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase (BCKD) enzyme complex, which catalyses the decarboxylation of the alpha-keto acids of leucine, isoleucine, and valine to their respective branched-chain acyl-CoAs. (medscape.com)
Quental et al identified a homozygous 1-bp deletion (117delC) in the BCKDHA gene (this gene codes for the alpha subunit of the BCKD enzyme complex, specifically E1) in Portuguese Gypsies and estimated the carrier frequency for this deletion to be as high as 1.4% (about 1 case per 71 live births). (medscape.com)
Pyruvate dehydrogenase (PDH) is an enzyme complex that catalyzes the conversion of pyruvate into acetyl-coA in vivo. (biomedcentral.com)
The defect in fatty alcohol oxidation in SLS is caused by the deficiency of microsomal fatty aldehyde dehydrogenase (FALDH, EC 1.2.1.48), a component of the fatty alcohol:NAD + oxidoreductase enzyme complex ( 6 , 7 ). (ajnr.org)
The cofactor regeneration system of leucine dehydrogenase (LeuDH) and glucose dehydrogenase (GDH) has showed great coupling catalytic efficiency in the synthesis of l -tle, however the multi-enzyme complex of GDH and LeuDH has never been constructed successfully. (biomedcentral.com)
It is the first report about the fusion of GDH and LeuDH as the multi-enzyme complex to synthesize l -tle and reach the highest space-time yield up to now. (biomedcentral.com)
The PDH complex also known as the pyruvate dehydrogenase complex is a multi-enzyme complex. (biotechfront.com)
All these intermediates of PDH catalysed reaction are not free but bound with an enzyme complex together called as the PDH complex. (biotechfront.com)
Pyruvate dehydrogenase is a multi-enzyme complex responsible for the generation of acetyl CoA from pyruvate for the Krebs cycle. (msdmanuals.com)
Complex II (Succinate-ubiquinone oxidoreductase) is an importnat enzyme complex for the tricarboxylic acid cycle and the aerobic respiratory chain of motochondria and procaryotic organisms (12, 24). (bvsalud.org)

what rxn is catalyzed by succinate dehydrogenase in the TCA? (flashcardmachine.com)
Complex II catalyzes the oxidation of succinate to fumarate (Succinate dehydrogenase: sdh) and transfers i. (bvsalud.org)
SQR consists of a membrane-peripheral heterodimer domain (often called succinate dehydrogenase) which is tightly bound to a membrane-spanning anchor, which is a cytochrome with one or two heme groups depending on the organism. (lu.se)
However, the E. coli cells did not show increased succinate dehydrogenase activity nor did the operon complement a sdhCDAB defective E. coli mutant [7]. (lu.se)

Activity of purified NAD-specific isocitrate dehydrogenase at modulator and substrate concentrations approximating conditions in mitochondria. (wikipathways.org)
what reaction is catalyzed by isocitrate dehydrogenase? (flashcardmachine.com)
isocitrate dehydrogenase (converts isocitrate to alpha ketoglutarate). (flashcardmachine.com)
how is isocitrate dehydrogenase regulated? (flashcardmachine.com)

24 pyruvate dehydrogenase has the coenzyme TPP (thiamin pyrophosphate) incorporated into it, 24 dihydrolipoyl transacetylase has lipoate and coenzyme A, and 12 dihydrolipoyl dehydrogenase has the coenzymes FAD and NAD+. (dadamo.com)

FAO is a complex enzyme which consists of two separate proteins that sequentially catalyze the oxidation of fatty alcohol to fatty aldehyde and fatty acid. (jci.org)
Intact SLS fibroblasts oxidized octadecanol to fatty acid at less than 10% of the normal rate, but oxidized free octadecanal normally, suggesting that the FALDH affected in SLS is chiefly involved in the oxidation of fatty alcohol to fatty acid. (jci.org)
These results show that the primary enzymatic defect in SLS is the FALDH component of the FAO complex, which leads to deficient oxidation of fatty aldehyde derived from fatty alcohol. (jci.org)
Pyruvate may be reduced to lactate in the cytoplasm or may be transported into the mitochondria for anabolic reactions, such as gluconeogenesis and lipogenesis, or for oxidation to acetyl CoA by the pyruvate dehydrogenase (PDH) complex (PDC). (bmj.com)
In the pyruvate dehydrogenase complex, it binds to the core of EC 2.3.1.12 and catalyzes oxidation of its dihydrolipoyl groups. (expasy.org)
One of these multienzymatic syntheses is the production of lactobionic acid which is obtained by oxidation of lactose in presence of flavocytochrome cellobiose dehydrogenase, CDH, as the first biocatalyst (see figure). (aiche.org)
Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) plays an important role in glycolysis and gluconeogenesis [ ( PUBMED:2716055 ) ] by reversibly catalysing the oxidation and phosphorylation of D-glyceraldehyde-3-phosphate to 1,3-diphospho-glycerate. (embl.de)
FALDH catalyzes the oxidation of medium- and long-chain fatty aldehydes, derived from fatty alcohols or not, to the corresponding carboxylic acids ( Fig 1 ). (ajnr.org)
FALDH catalyzes the oxidation of long-chain fatty aldehydes (here, octadecanal) to the corresponding carboxylic acid. (ajnr.org)

A component of the multienzyme 2-oxo-acid dehydrogenase complexes. (expasy.org)
It is a component of the multienzyme 3-methyl-2-oxobutanoate dehydrogenase complex in which multiple copies of it are bound to a core of molecules of EC 2.3.1.168 , dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which also binds multiple copies of EC 1.8.1.4 , dihydrolipoyl dehydrogenase. (enzyme-database.org)

Finally, the space-time yield of l -tle catalyzing by GDH-R3-LeuDH whole cells could achieve 2136 g/L/day in a 200 mL scale system under the optimal catalysis conditions (pH 9.0, 30 ° C, 0.4 mM of NAD + and 500 mM of a substrate including trimethylpyruvic acid and glucose). (biomedcentral.com)
Analysis of the structure and substrate binding of Phormidium lapideum alanine dehydrogenase. (expasy.org)

Nucleic Acids Res. (nih.gov)

Through a series of chemical reactions of pyruvate decarboxylation, the glycolytic pathway (the final product is pyruvate) and the tricarboxylic acid cycle (the initial reactant is acetyl-coA) can be effectively connected [ 7 ]. (biomedcentral.com)
As an important intermediate metabolite, acetyl-coA not only participates in tricarboxylic acid cycle as well as the glyoxylate cycle but also provides carbon source for the synthesis of mycolic acid and lipids [ 8 , 9 ]. (biomedcentral.com)

Lipoic acid. (biotechfront.com)
Arsenite binds to thiol Which is sulfhydryl-SH group of lipoic acid and makes it unavailable to serve as a cofactor. (biotechfront.com)

Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. (thermofisher.com)
The analysis of mycolic acid (MA) using LC-MS indicated deficiency of alpha-MA and epoxy-MA in aceE -mut strain whereas complementation of the aceE -mut with a wild-type aceE gene restored the composition of MA. (biomedcentral.com)
Inherited or acquired alcoholics deficiency of PDH causes lactic acidosis due to rapid conversion of accumulated pyruvate into lactic acid. (biotechfront.com)
Deficiency results in elevation of pyruvate and thus elevation of lactic acid levels. (msdmanuals.com)
Diagnosis of pyruvate dehydrogenase deficiency is confirmed by enzyme analysis of skin fibroblasts, DNA testing, or both. (msdmanuals.com)
There is no clearly effective treatment for pyruvate dehydrogenase deficiency, although a low-carbohydrate or ketogenic diet and dietary thiamin supplementation have been beneficial for some patients. (msdmanuals.com)

Deficient activity of the fatty aldehyde dehydrogenase component of fatty alcohol:NAD+ oxidoreductase in cultured fibroblasts. (jci.org)

Mutations in the DLD gene impair the function of the DLD enzyme, which prevents the three enzyme complexes from functioning properly. (medlineplus.gov)
Gene Trp-T encodes an enzyme that converts tryptophan to indole-3-pyruvic acid (I3PA), which is then converted to IAA by an enzyme encoded by the gene YUC. (onlinemathlearning.com)
ProComp leverages, and cross references, entries in existing protein-centric informatics resources, including the protein complexes that are represented in the Cellular Component branch of the Gene Ontology. (biomedcentral.com)
Over all, this study indicates that aceE gene plays a significant role in the mycolic acid synthesis and affects the colony morphology, biofilm formation of M. smegmatis and bacteria invasion of macrophage. (biomedcentral.com)
In sickle cell anemia, a point mutation on the β-globin gene results in glutamic acid substituting for valine at position 6 of the amino acid sequence. (medscape.com)

The conversion of pyruvate into acetyl-CoA is referred to as the Pyruvate Dehydrogenase Reaction. (dadamo.com)
The pyruvate dehydrogenase complex (PDC) catalyzes a very important metabolic reaction: the conversion of pyruvate to acetyl-CoA [ Pyruvate Dehydrogenase Reaction ]. (blogspot.com)

Selective inhibitors of the parasite lactate dehydrogenase (LDH), central to NAD(+) regeneration, therefore potentially provide a route to new antimalarial drugs directed against a novel molecular target. (rcsb.org)
A series of heterocyclic, azole-based compounds are described that preferentially inhibit P. falciparum LDH at sub-micromolar concentrations, typically at concentrations about 100-fold lower than required for human lactate dehydrogenase inhibition. (rcsb.org)
Lactate dehydrogenase (LDH) release was also measured as an indicator of cytotoxicity. (cdc.gov)

To determine which enzymatic component of FAO was deficient in SLS, we assayed fatty aldehyde dehydrogenase (FALDH) and fatty alcohol dehydrogenase in cultured fibroblasts from seven unrelated SLS patients. (jci.org)
All SLS cells were selectively deficient in the FALDH component of FAO, and had normal activity of fatty alcohol dehydrogenase. (jci.org)
alcohol dehydrogenase 5 (class III), chi. (wikigenes.org)

Mutations in E3 cause additional deficiencies of pyruvate and alpha-ketoglutarate dehydrogenases. (medscape.com)

The pyruvate dehydrogenase (PDH) multienzyme complex (PDC). (bmj.com)

Recall that the E 3 subunit of PDC is shared with 2-oxo acid dehydrogenase, an enzyme required for the breakdown of branched chain amino acids. (blogspot.com)
Differing epitope selection of experimentally-induced and natural antibodies to a disease-specific autoantigen, the E2 subunit of pyruvate dehydrogenase complex (PDC-E2). (edu.au)

I. Isolation, purification, and partial characterization of bovine liver α-ketoisocaproic:α-keto-β-methylvaleric acid dehydrogenase. (enzyme-database.org)
Pettit, F.H., Yeaman, S.J. and Reed, L.J. Purification and characterization of branched chain α-keto acid dehydrogenase complex of bovine kidney. (enzyme-database.org)

what reaction is catalyzed by alpha ketoglutarate dehydrogenase? (flashcardmachine.com)
this protein is involved in the modification of additional proteins that aid in energy production in mitochondria, including the pyruvate dehydrogenase complex and the alpha-ketoglutarate dehydrogenase complex. (medlineplus.gov)

Enzyme assays were conducted to measure dehydrogenase and acid phosphatase activities, and similar assays were performed for fluorescein diacetate (FDA) activity to assess potential degradation of complex organics. (usda.gov)
• PDH phosphatase which is responsible to maintain PDH complex an active state is promoted by Calcium(Ca²+) Magnesium( Mg²+) and mainly insulin. (biotechfront.com)

While the field of microbiology has adapted to the study of complex microbiomes via modern meta-omics techniques, we have not updated our basic knowledge regarding the quantitative levels of DNA, RNA and protein molecules within a microbial cell, which ultimately control cellular function. (nature.com)
Representing species-specific proteins and protein complexes in ontologies that are both human- and machine-readable facilitates the retrieval, analysis, and interpretation of genome-scale data sets. (biomedcentral.com)
Because proteins are often functional only as members of stable protein complexes, the PRO Consortium, in collaboration with existing protein and pathway databases, has launched a new initiative to implement logical and consistent representation of protein complexes. (biomedcentral.com)
We describe here how the PRO Consortium is meeting the challenge of representing species-specific protein complexes, how protein complex representation in PRO supports annotation of protein complexes and comparative biology, and how PRO is being integrated into existing community bioinformatics resources. (biomedcentral.com)
PRO is a unique database resource for species-specific protein complexes. (biomedcentral.com)
PRO facilitates robust annotation of variations in composition and function contexts for protein complexes within and between species. (biomedcentral.com)
Logical and semantic access to related protein forms is critical for advancing bioinformatics approaches to representing, modeling, and reasoning about complex biological systems at the genomic and cellular level [ 1 ]. (biomedcentral.com)
ProComp, the focus of this manuscript, represents multi-protein complexes, with an initial (but not exclusive) emphasis on protein components of complexes in mouse and human. (biomedcentral.com)
Protein complexes may have other associated non-protein prosthetic groups, such as nucleotides, metal ions or other small molecules. (biomedcentral.com)
Protein complexes are distinguished from protein-protein interactions in that they are continuant entities, i.e. they endure or continue to exist through time. (biomedcentral.com)
The explicit representation of protein complexes in PRO--defining each member of the complex at the level of its isoform, variant, or modified form--provides the ability to represent complex biological knowledge as it is emerging in the experimental research community in structures that are both human readable and accessible to algorithmic approaches. (biomedcentral.com)
In the GO, types of protein complexes are defined in terms of constituent macromolecule classes and the function(s) that the complexes carry out. (biomedcentral.com)
A major contribution of PRO as a protein biology community informatics resource is that it provides a formal ontological structure with foundation in Basic Formal Ontology http://www.ifomis.org/bfo/ to describe types of protein complexes and gives these types unique, permanent identifiers http://www.obofoundry.org/id-policy.shtml . (biomedcentral.com)
Many of the proteins involved in this process require Fe-S clusters to function, including protein complexes called complex I, complex II, and complex III. (medlineplus.gov)

Cytochrome c oxidase (complex IV) catalyses the reduction of molecular oxygen to water and ATP synthase (complex V) generates ATP from ADP. (bmj.com)

Maple syrup urine disease (MSUD), also known as branched-chain ketoaciduria, is an aminoacidopathy due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. (medscape.com)
It acts not only on 3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and ( S )-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids that derive from the action of transaminases on valine, leucine and isoleucine. (enzyme-database.org)
Among those biocatalysts, leucine dehydrogenase (LeuDH, EC 1.4.1.9) exhibited an outstanding conversion efficiency and enantioselectivity which became the main method of l -tle synthesis in the market [ 11 ]. (biomedcentral.com)

These are formed in the liver and kidneys from the breakdown of fatty acids and a few amino acids. (medscape.com)
In the liver, when levels of circulating fatty acids are high, the production of acetyl-CoA from fat breakdown exceeds the cellular energy requirements. (dadamo.com)

The role of citric acid in intermediate metabolism in animal tissues. (wikipathways.org)
Bowden, J.A. and Connelly, J.L. Branched chain α-keto acid metabolism. (enzyme-database.org)
The phenotype results from a defect in amino acid metabolism and not from a defect in pyruvate dehydrogenase. (blogspot.com)

The resulting hydroxyethyl-TPP complex reacts with oxidised lipoamide (LipS 2 ), the prosthetic group of dihydrolipoamide transacetylase (E 2 ), to form acetyl lipoamide. (bmj.com)
Acetyl-CoA reacts with the four carbon carboxylic acid, oxaloacetate--to form the six carbon carboxylic acid, citrate. (citizendium.org)
An acetyl coenzyme A mainly derived from fats which are triglycerides, fatty acids, cholesterol. (biotechfront.com)

Biocatalysis is now offering a range of new catalytic options for industrial complex syntheses. (aiche.org)

Pyruvate Dehydrogenase with TPP as coenzyme, decarboxylates pyruvate to Hydroxyethyl TPP (thiamine pyrophosphate). (biotechfront.com)

In fat catabolism , triglycerides are hydrolyzed to break them into fatty acids and glycerol . (citizendium.org)
An exception to this rule is Glycerol and Propionic acid which are derived from fats, but they can be converted to glucose. (biotechfront.com)

Nervous and muscle tissues preferentially oxidize ketones over fatty acids, glucose, or amino acids under normal conditions. (medscape.com)
As a whole five ATP are released in the PDH complex and from two moles of pyruvate which is formed from glucose by glycolysis . (biotechfront.com)

In the following years, Dancis et al identified the pathogenetic compounds as branched-chain amino acids and their corresponding alpha-keto acids. (medscape.com)
Organic dusts in dairy farm environments are complex, polydisperse mixtures of toxic and immunogenic compounds. (cdc.gov)

Affected infants often have severe brain dysfunction (encephalopathy) and elevated levels of a chemical called lactic acid in the body (lactic acidosis). (medlineplus.gov)

[ 4 ] In 1960, Dancis et al demonstrated that the enzymatic defect in maple syrup urine disease was at the level of the decarboxylation of the branched-chain amino acids. (medscape.com)

During the reaction, the reduced state of CDH is oxidized again by the utilization of an intermediary redox mediator that in this cases is 2,2'-azinobis(3-ethylbenzothiazoline-6-sulfonic acid) (ABTS). (aiche.org)

We have discovered that erythrocyte frataxin is in fact a novel isoform of frataxin (isoform E) with 135-amino acids and an N-terminally acetylated methionine residue. (nih.gov)

MMDB and VAST+: tracking structural similarities between macromolecular complexes. (nih.gov)

The carboxyl group of mycolic acids is vertically covalently linked to the hydroxyl group of arabino-galactan by ester bond, arabino-galactan is linked to the peptidoglycan layer by phospholipid bond, whereas other glycolipids and free lipids are regularly distributed in the thicker layer of mycolic acids [ 3 , 4 , 5 ]. (biomedcentral.com)

The enzyme consists of 60 subunits: 24 pyruvate dehydrogenase, 24 dihydrolipoyl transacetylase, and 12 dihydrolipoyl dehydrogenase (commonly denoted E1, E2, and E3). (dadamo.com)

Anatomy8

Organisms8

Diseases7

Chemicals and Drugs104

Analytical, Diagnostic and Therapeutic Techniques and Equipment7

Phenomena and Processes23

Disciplines and Occupations1

Technology, Industry, Agriculture1

Information Science3

Dihydrolipoamide dehydrogenase12

Enzymes19

Citric acid16

Pyruvate dehydrogenase and alpha-ketoglutarate1

NADH3

Mitochondrial5

Glycine-cleavage system1

Enzyme complex16

Succinate dehydrogenase4

Isocitrate dehydrogenase4

Lipoate1

Oxidation9

Component of the multienzyme2

Substrate2

Nucleic Aci1

Tricarboxylic acid2

Lipoic2

Deficiency6

Oxidoreductase1

Gene5

Conversion of pyruvate2

Lactate dehydrogenase3

Alcohol Dehydrogenase3

Mutations1

Multienzyme1

Subunit2

Bovine2

Alpha ketoglutarate2

Phosphatase2

Protein14

Synthase1

Leucine3

Liver2

Metabolism3

Acetyl3

Catalytic1

Thiamine1

Glycerol2

Glucose2

Compounds2

Lactic acidosis1

Decarboxylation1

Reaction1

Isoform1

Macromolecular1

Lipids1

Consists1