SubunitKinaseEnzymesEnzymePatients with pyruvate dehydrogenase compMitochondrial pyruvate dehydroCitric acidPhosphorylation of pyruvate dehydrogenaseAlpha-ketoacid dehydrogenase complexesHeart pyruvate dehydrogenaseHuman pyruvateThiamine pyrophosphateMetabolicMitochondriaProteinsPrimary lactic acidosisOxidative decarboxylationLactateDihydrolipoyl dehydrogenaseCatalyticConvertsPDHA1Carboxylase deficiencyInactivationRegulationMutationsMultienzymeCytosolE3BPPDCDGlutamateMammalianKinasesStructuralDecarboxylaseProtein complexesVitroMoleculesMetabolism disordersReactionDisordersGeneAminoBiochemicalCarnitineFunctionalLactic acid
Subunit13
- The reaction catalysed by pyruvate dehydrogenase complex is: The E1 subunit, called the pyruvate dehydrogenase subunit, has a structure that consists of two chains (an "ɑ" and "ꞵ" chain). (wikipedia.org)
- The E3 subunit, called the dihydrolipoyl dehydrogenase enzyme, is characterized as a homodimer protein wherein two cysteine residues, engaged in disulfide bonding, and the FAD cofactor in the active site facilitate its main purpose as an oxidizing catalyst. (wikipedia.org)
- An auxiliary protein unique to most eukaryotes is the E3 binding protein (E3BP), which serves to bind the E3 subunit to the PDC complex. (wikipedia.org)
- Two elements regulate the complex: an E1 kinase and a phospho-E1 phosphatase, which phosphorylate and dephosphorylate, respectively, three serine residues in the E1 α subunit, resulting in the deactivation and activation, respectively, of the complex (2) . (nature.com)
- Most cases result from mutations in the X-linked gene for the pyruvate dehydrogenase E1α subunit (PDHA1) while a few cases result from mutations in genes for E1β (PDHB), E2 (DLAT), E3 (DLD) and E3BP (PDHX) subunits or PDH-phosphatase (PDP1). (nih.gov)
- Methods To understand the role of pyruvate dehydrogenase complex in T cell differentiation, we generated mice deficient in T cell pyruvate dehydrogenase E1A ( Pdha ) subunit using a CD4-cre recombinase-based strategy. (nih.gov)
- Vitamin cofactor for E2 subunit of the pyruvate dehydrogenase complex. (medscape.com)
- Mild phenotype in a male with pyruvate dehydrogenase complex deficiency associated with novel hemizygous in-frame duplication of the E1a subunit gene (PDHA1). (medscape.com)
- Pyruvate dehydrogenase complex deficiency due ubiquitination and proteasome-mediated degradation of the E1beta subunit. (medscape.com)
- The most common deficiency involves the E1 subunit, a heterotetramer consisting of two alpha and two beta subunits with the defect located on the alpha subunit (E1 α ), which contains several serine phosphorylation sites and seems to be involved in the regulation of the whole complex. (mhmedical.com)
- In HD, the complex II subunit SDHB was lowered although not sufficiently to affect complex II activity. (nature.com)
- We used as a variable the differential expression of MDM2 protein as a function of cell density to identify Nutlin-3 responsive MDM2-binding proteins that are perturbed independent of cell density using SWATH-MS. Dihydrolipoamide dehydrogenase, the E3 subunit of the mitochondrial pyruvate dehydrogenase complex, was one of two Nutlin-3 perturbed proteins identified fours hour posttreatment at two cell densities. (strath.ac.uk)
- Pyruvate dehydrogenase (PDH) complex deficiency is one of the important causes of congenital lactic acidemia and mostly due to defect in the α subunit of PDH (E1α), of which gene is located on the X chromosome. (tokushima-u.ac.jp)
Kinase6
- Other associated proteins control the activity of the complex: pyruvate dehydrogenase phosphatase turns on (activates) the complex, while pyruvate dehydrogenase kinase turns off (inhibits) the complex. (medlineplus.gov)
- In addition, there are other subunits, E3 binding protein (E3-BP) and two complex-regulating enzymes: pyruvate dehydrogenase kinase, which inactivates the complex, and pyruvate dehydrogenase phosphatase, which reactivates the complex. (mhmedical.com)
- 20. Pyruvate dehydrogenase kinase activity of pig heart pyruvate dehydrogenase (E1 component of pyruvate dehydrogenase complex). (nih.gov)
- Metabolism of PEP to pyruvate by pyruvate kinase (PK) generates 1 molecule of adenosine triphosphate (ATP) via substrate-level phosphorylation. (hmdb.ca)
- This gene encodes a member of the pyruvate dehydrogenase kinase family. (antibodies-online.com)
- PDH activity is inhibited by pyruvate dehydrogenase kinase (PDK). (me-pedia.org)
Enzymes11
- Pyruvate dehydrogenase complex (PDC) is a complex of three enzymes that converts pyruvate into acetyl-CoA by a process called pyruvate decarboxylation. (wikipedia.org)
- Molecular structure of a 9-MDa icosahedral pyruvate dehydrogenase subcomplex containing the E2 and E3 enzymes using cryoelectron microscopy. (nih.gov)
- Essential cofactor for several important enzymes, including an alternative pathway for pyruvate. (medscape.com)
- The pyruvate dehydrogenase complex is made up of multiple copies of several enzymes called E1, E2, and E3, each of which performs part of the chemical reaction that converts pyruvate to acetyl-CoA. (medlineplus.gov)
- A deficiency in any one of the enzymes involved in pyruvate metabolism leads to one of many disorders. (msdmanuals.com)
- Many enzymes are involved in pyruvate metabolism. (msdmanuals.com)
- This disorder is caused by a lack of a group of enzymes needed to process pyruvate. (msdmanuals.com)
- The a-keto acid dehydrogenase complexes are composed of multiple copies of three enzymes that catalyze a total of five reactions (Figure 5.1). (taylorfrancis.com)
- To help define in molecular detail the catalytic mechanism of these enzymes, we report here the high resolution crystal structure of wild-type murine carnitine acetyltransferase (CrAT) in a ternary complex with its substrates acetyl-CoA and carnitine, and the structure of the S554A/M564G double mutant in a ternary complex with the substrates CoA and hexanoylcarnitine. (proteopedia.org)
- One of the enzymes that belong to 2-OADHC is pyruvate dehydrogenase complex (PDC). (medscape.com)
- These two metabolic pathways liberate electrons that are transported through four respiratory chain enzymes (complexes I-IV) embedded within the inner membrane of mitochondria. (mhmedical.com)
Enzyme19
- This multi-enzyme complex is related structurally and functionally to the oxoglutarate dehydrogenase and branched-chain oxo-acid dehydrogenase multi-enzyme complexes. (wikipedia.org)
- At this point, the lipoate-thioester functionality is translocated into the dihydrolipoyl transacetylase (E2) active site, where a transacylation reaction transfers the acetyl from the "swinging arm" of lipoyl to the thiol of coenzyme A. This produces acetyl-CoA, which is released from the enzyme complex and subsequently enters the citric acid cycle. (wikipedia.org)
- The binary complexes of the enzyme with CoA and Lip(SH)2 were determined at 2.6- and 3.0-A resolutions, respectively. (rcsb.org)
- Important cofactor for the pyruvate dehydrogenase complex E1 enzyme. (medscape.com)
- Dichloroacetate sodium is the only drug found to activate the enzyme complex. (medscape.com)
- The E1 enzyme, also called pyruvate dehydrogenase, is composed of four parts (subunits): two alpha subunits (called E1 alpha) and two beta subunits (called E1 beta). (medlineplus.gov)
- Mutations in the genes that provide instructions for E1 beta (the PDHB gene), the E2 enzyme (the DLAT gene), E3 binding protein (the PDHX gene), and pyruvate dehydrogenase phosphatase (the PDP1 gene) have been identified in people with this condition. (medlineplus.gov)
- Mammalian pyruvate dehydrogenase complex (PDC) is a key multi-enzyme assembly that is responsible for glucose homeostasis maintenance and conversion of pyruvate into acetyl-CoA. (ox.ac.uk)
- Pyruvate dehydrogenase (PDH) is an enzyme complex consisting of three catalytic subunits, pyruvate dehydrogenase (E1, a heterotetramer, α 2 β 2), dihydrolipoamide acyltransferase (E2, a monomer), and dihydrolipoamide dehydrogenase (E3, a dimer), and two cofactors, thiamine pyrophosphate and lipoic acid. (mhmedical.com)
- The enzyme complex converts pyruvate-after it enters the mitochondria-into acetyl-CoA, one of two essential substrates (oxaloacetate being the other) in the production of citrate. (mhmedical.com)
- The enzyme defect causes more pyruvate to be metabolized to lactate and leads to lactic acidosis. (mhmedical.com)
- Four enzyme complexes requiring lipoic acid are located in the mitochondrial matrix space of higher eurkaryotes. (taylorfrancis.com)
- Detailed analyses suggest that these structures may be good mimics for the Michaelis complexes for the forward and reverse reactions of the enzyme, representing the first time that such complexes of CrAT have been studied in molecular detail. (proteopedia.org)
- Pyruvate dehydrogenase is a multi-enzyme complex responsible for the generation of acetyl CoA from pyruvate for the Krebs cycle. (merckmanuals.com)
- Diagnosis of pyruvate dehydrogenase deficiency is confirmed by enzyme analysis of skin fibroblasts, DNA testing, or both. (merckmanuals.com)
- Pyruvate carboxylase is an enzyme important for gluconeogenesis from pyruvate and alanine generated in muscle. (merckmanuals.com)
- In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. (nih.gov)
- Evidence that high frequency stimulation influences the phosphorylation of pyruvate dehydrogenase and that the activity of this enzyme is linked to mitochondrial calcium sequestration. (ox.ac.uk)
- Pyruvate dehydrogenase or PDH is an enzyme that is part of the citric acid cycle . (me-pedia.org)
Patients with pyruvate dehydrogenase comp2
- Molecular characterization of 82 patients with pyruvate dehydrogenase complex deficiency. (nih.gov)
- However, administration of all of these cofactors to all patients with pyruvate dehydrogenase complex deficiency is typical in order to optimize pyruvate dehydrogenase complex function. (medscape.com)
Mitochondrial pyruvate dehydro1
- The encoded protein phosphorylates pyruvate dehydrogenase, down-regulating the activity of the mitochondrial pyruvate dehydrogenase complex. (antibodies-online.com)
Citric acid3
- Acetyl-CoA may then be used in the citric acid cycle to carry out cellular respiration, and this complex links the glycolysis metabolic pathway to the citric acid cycle. (wikipedia.org)
- In humans, PDC converts pyruvate to acetyl-CoA, which leads into the citric acid cycle located in the mitochondrion. (gla.ac.uk)
- Mitochondria perform multiple vital biochemical functions, including breakdown of fatty acids through β-oxidation and catabolism of pyruvate derived from glycogen via the Krebs or citric acid cycle ( Figure 24-1 ). (mhmedical.com)
Phosphorylation of pyruvate dehydrogenase1
- 8. Reversible phosphorylation of pyruvate dehydrogenase in rat skeletal-muscle mitochondria. (nih.gov)
Alpha-ketoacid dehydrogenase complexes2
Heart pyruvate dehydrogenase1
Human pyruvate2
- Solution structure and characterisation of the human pyruvate dehydrogenase complex core assembly. (ox.ac.uk)
- The crystal structure of dihydrolipoamide dehydrogenase and dihydrolipoamide dehydrogenase-binding protein (didomain) subcomplex of human pyruvate dehydrogenase complex. (yeastrc.org)
Thiamine pyrophosphate2
- Initially, pyruvate and thiamine pyrophosphate (TPP or vitamin B1) are bound by pyruvate dehydrogenase subunits. (wikipedia.org)
- E1, one of the subunits of the complex, is a thiamine pyrophosphate-dependent pyruvate decarboxylase. (nature.com)
Metabolic4
- A diagnosis of pyruvate dehydrogenase complex deficiency was made on the basis of metabolic and molecular genetic studies. (nih.gov)
- Background Modulation of metabolic flux through pyruvate dehydrogenase complex (PDC) plays an important role in T cell activation and differentiation. (nih.gov)
- 12. Metabolic and oncogenic adaptations to pyruvate dehydrogenase inactivation in fibroblasts. (nih.gov)
- Robert A. Harris, PhD , distinguished professor emeritus, Showalter Professor emeritus and former chair of the Department of Biochemistry and Molecular Biology at Indiana University School of Medicine, will present, "The Roles of Pyruvate Dehydrogenase Kinases in Metabolic Homeostasis and Diseases. (iu.edu)
Mitochondria5
- The beneficial effects of DCA treatment are thought to arise from an increase in the flux of pyruvate to mitochondria and a decrease in serum lactate levels mediated by the stimulation of PDHC, as demonstrated by experiments involving chronic daily supplementation in rats (10) . (nature.com)
- This video shows a molecular view of the reactions that take place inside the pyruvate dehydrogenase complex, a protein machine found in the cell's powerhouse, the mitochondria. (nih.gov)
- 9. Phosphorylation-mediated changes in pyruvate dehydrogenase activity influence pyruvate-supported calcium accumulation by brain mitochondria. (nih.gov)
- The protein encoded by this gene is the NADP(+)-dependent isocitrate dehydrogenase found in the mitochondria. (cancerindex.org)
- This coincides with an increase in MDM2/dihydrolipoamide dehydrogenase complexes in the nucleus that was further enhanced by the nuclear export inhibitor Leptomycin B. The data suggest one therapeutic impact of MDM2 drugs might be on the early perturbation of specific protein-protein interactions within the mitochondria. (strath.ac.uk)
Proteins4
- The genes involved in pyruvate dehydrogenase deficiency each provide instructions for making a protein that is a component of a group of proteins called the pyruvate dehydrogenase complex. (medlineplus.gov)
- In addition, other proteins included in the complex ensure its proper function. (medlineplus.gov)
- One of these proteins, E3 binding protein, attaches E3 to the complex and provides the correct structure for the complex to perform its function. (medlineplus.gov)
- We are involved in several collaborative applications in various fields including immunological protein complexes, viral proteins, membrane receptor complexes, and eye lens crystallins. (nih.gov)
Primary lactic acidosis1
- Pyruvate dehydrogenase complex (PDHc) deficiencies are an important cause of primary lactic acidosis. (nih.gov)
Oxidative decarboxylation2
- This multienzyme complex plays an important role in the irreversible oxidative decarboxylation of pyruvate to acetyl-CoA. (nature.com)
- Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. (cancerindex.org)
Lactate4
- This decreases lactate production and promotes pyruvate oxidation. (medscape.com)
- Debray FG, Mitchell GA, Allard P, Robinson BH, Hanley JA, Lambert M. Diagnostic accuracy of blood lactate-to-pyruvate molar ratio in the differential diagnosis of congenital lactic acidosis. (medscape.com)
- The most important laboratory test for the diagnosis of a possible PDHCD is the measurement of plasma and cerebrospinal fluid (CSF) lactate and pyruvate. (mhmedical.com)
- With this DNA diagnostic system we could diagnose 4 female patients as PDH E1α deficiency from 14 female patients who were suspected PDH complex deficiency from the clinical features and concentrations of lactate and pyruvate in the blood but showed normal PDH complex activity in their cultured cells. (tokushima-u.ac.jp)
Dihydrolipoyl dehydrogenase2
- The dihydrolipoate, still bound to a lysine residue of the complex, then migrates to the dihydrolipoyl dehydrogenase (E3) active site, where it undergoes a flavin-mediated oxidation, identical in chemistry to disulfide isomerase. (wikipedia.org)
- Up to 24 copies of pyruvate dehydrogenase (E1) and 12 molecules of dihydrolipoyl dehydrogenase (E3) bind to the outside of the E2 core. (wikipedia.org)
Catalytic4
- The catalytic domain of dihydrolipoyl transacetylase (E2pCD) forms the core of the pyruvate dehydrogenase multienzyme complex and catalyzes the acetyltransferase reaction using acetylCoA as acetyl donor and dihydrolipoamide (Lip(SH)2) as acceptor. (rcsb.org)
- E2 has a multi-domain structure where the C-terminal catalytic domains (CD) of several E2 subunits interact to form the core of the complex. (gla.ac.uk)
- Molecular architecture and mechanism of an icosahedral pyruvate dehydrogenase complex: a multifunctional catalytic machine. (nih.gov)
- 2003. Age-related increase in 4-hydroxynonenal adduction to rat heart alpha-ketoglutarate dehydrogenase does not cause loss of its catalytic activity. . (oregonstate.edu)
Converts1
- The pyruvate dehydrogenase complex converts a molecule called pyruvate, which is formed from the breakdown of carbohydrates, into another molecule called acetyl-CoA. (medlineplus.gov)
PDHA13
- Four novel PDHA1 mutations in pyruvate dehydrogenase deficiency. (medscape.com)
- Mutations in the gene that provides instructions for making E1 alpha, the PDHA1 gene, are the most common cause of pyruvate dehydrogenase deficiency, accounting for approximately 80 percent of cases. (medlineplus.gov)
- As with PDHA1 gene mutations, changes in these other genes lead to a reduction of pyruvate dehydrogenase complex activity. (medlineplus.gov)
Carboxylase deficiency2
- In addition to pyruvate carboxylase deficiency, defects in the mitochondrial chain of electron transport or in gluconeogenesis should be considered. (mhmedical.com)
- There is no effective treatment for pyruvate carboxylase deficiency, but some patients with primary deficiency and all those with secondary deficiencies should be given biotin supplementation 5 to 20 mg orally once a day. (merckmanuals.com)
Inactivation1
- However, some female patients, who are heterozygous for the mutant allele, cannot be diagnosed only by the assay of PDH complex activity, because of the skewed X-chromosome inactivation in cultured cells. (tokushima-u.ac.jp)
Regulation2
- Regulation of the activity of the pyruvate dehydrogenase complex of Escherichia coli. (wikidata.org)
- His research has focused on the regulation of pyruvate dehydrogenase complex by kinases in starvation, diabetes and cancer. (iu.edu)
Mutations3
- Some evidence suggests that high doses of thiamine may be most effective in some mutations causing thiamine-responsive pyruvate dehydrogenase complex deficiency. (medscape.com)
- Although it is unclear how mutations in each of these genes affect the complex, reduced functioning of one component of the complex appears to impair the activity of the whole complex. (medlineplus.gov)
- Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. (nih.gov)
Multienzyme2
- Extended polypeptide linkers establish the spatial architecture of a pyruvate dehydrogenase multienzyme complex. (nih.gov)
- A multienzyme complex responsible for the formation of ACETYL COENZYME A from pyruvate. (bvsalud.org)
Cytosol1
- 17. Engineering acetyl coenzyme A supply: functional expression of a bacterial pyruvate dehydrogenase complex in the cytosol of Saccharomyces cerevisiae. (nih.gov)
E3BP1
- E3 is a dihydrolipoamide dehydrogenase, and E3BP or protein X mediates the interaction between E2 and E3. (nature.com)
PDCD2
- The cases of pyruvate dehydrogenase complex deficiency (PDCD) that are responsive to these cofactors respond to supplementation, especially thiamine. (medscape.com)
- Project Abstract/Summary Pyruvate dehydrogenase complex (PDC) deficiency (PDCD) is a rare disease of mitochondrial energy failure in which the life expectancy of affected children is severely truncated from unrelenting lactic acidosis and/or from progressive neurological and neuromuscular degeneration. (sbir.gov)
Glutamate1
- Pickel, Virginia M. / Regional and cellular distribution of glutamate dehydrogenase and pyruvate dehydrogenase complex in brain : Implications for neurodegenerative disorders . (nyu.edu)
Mammalian1
- The mammalian PDH complex differs from that in E. coli and from the other mammalian alpha-keto acid dehydrogenases by the presence of a 53-kD protein called protein X. Component X binds to the E3 (MIM 238331) component of the PDH complex (Robinson et al. (novusbio.com)
Kinases1
- 15. Differing roles of pyruvate dehydrogenase kinases during mouse oocyte maturation. (nih.gov)
Structural2
- Dichloroacetate (DCA) is a structural analog of pyruvate that has been recommended for the treatment of primary lactic acidemia, particularly in patients with pyruvate dehydrogenase (PDHC) deficiency. (nature.com)
- The structural analysis of macromolecular complexes and assemblies is one of the major frontiers of structural biology, and an area where NIH intramural scientists are making important contributions. (nih.gov)
Decarboxylase1
- and inhibited when a specific serine residue in the pyruvate decarboxylase is phosphorylated by ATP . (bvsalud.org)
Protein complexes6
- The Laoratory of Dynamics of Macromolecular Assembly develops biophysical methods to study protein interactions and the assembly of multi-protein complexes. (nih.gov)
- Hallmarks of multi-protein complexes are multi-valent interactions and cooperativity. (nih.gov)
- Complementary to crystallographic techniques, such solution interaction studies can provide information on the assembly principles of structurally polymorph multi-protein complexes. (nih.gov)
- Proximity ligation identified rearrangements of cellular protein-protein complexes in situ. (strath.ac.uk)
- In response to Nutlin-3, a reduction of dihydrolipoamide dehydrogenase/dihydrolipoamide acetyltransferase protein complexes highlighted a disruption of the pyruvate dehydrogenase complex. (strath.ac.uk)
- This methodology forms a blueprint for biomarker discovery that can identify rearrangements of MDM2 protein-protein complexes in drug-treated cells. (strath.ac.uk)
Vitro1
- Monomethylarsonous acid (MMA(III)) and arsenite: LD(50) in hamsters and in vitro inhibition of pyruvate dehydrogenase. (nih.gov)
Molecules2
- However, little is known about the mechanisms of transporters and permeation of complex molecules or ions. (nih.gov)
- A shift in these or their expression means a shift towards higher glucose, lower pyruvate, lower Acetyl Co-A, and fewer energy-rich molecules produced in the cell to do work. (me-pedia.org)
Metabolism disorders2
- In many pyruvate metabolism disorders, both parents of the affected child carry a copy of the abnormal gene. (msdmanuals.com)
- Symptoms of pyruvate metabolism disorders may develop any time between early infancy and late adulthood. (msdmanuals.com)
Reaction5
- Pyruvate decarboxylation is also known as the "pyruvate dehydrogenase reaction" because it also involves the oxidation of pyruvate. (wikipedia.org)
- In this abortive ternary complex, CoA adopts a helical conformation with two intramolecular hydrogen bonds and the reactive sulfur of the pantetheine arm positioned 12 A away from the active site residues involved in the transferase reaction. (rcsb.org)
- With decreased function of this complex, pyruvate builds up and is converted in another chemical reaction to lactic acid. (medlineplus.gov)
- The mechanism of the pyruvate dehydrogenase reaction is wonderfully complex, more so than is suggested by its relatively simple stoichiometry. (spinnerstownhotel.com)
- It catalyzes the reaction that transforms pyruvate into acetyl-CoA , a process called pyruvate decarboxylation . (me-pedia.org)
Disorders2
- Although a relapsing-remitting course can be mimicked by, for example, various mitochondrial disorders (pyruvate dehydrogenase complex deficiency, Leigh disease, MELAS) Fabry disease, and X-linked Charcot-Marie-Tooth disease, awareness of these red flags is especially important for clinicians evaluating patients meeting criteria for (or carrying tentative diagnoses of) primary progressive multiple sclerosis, as most other heritable mimics of multiple sclerosis are more likely to exhibit a progressive disease course. (medscape.com)
- Some specific pyruvate disorders are helped by changes in diet. (msdmanuals.com)
Gene3
- 14. Prolonged hypoxia decreases nuclear pyruvate dehydrogenase complex and regulates the gene expression. (nih.gov)
- We explore how whole-genome duplications (WGDs) may have given rise to complex innovations in cellular networks, innovations that could not have evolved through sequential single-gene duplications. (hindawi.com)
- The PDHX gene encodes component X of the pyruvate dehydrogenase (PDH) complex. (novusbio.com)
Amino1
- A magnesium ion forms a 4-coordinate complex with three, polar amino acid residues (Asp, Asn, and Tyr) located on the alpha chain, and the thiamine diphosphate (TPP) cofactor directly involved in decarboxylation of the pyruvate. (wikipedia.org)
Biochemical2
- The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients. (medscape.com)
- Intermittent-relapsing pyruvate dehydrogenase complex deficiency: a case with clinical, biochemical, and neuroradiological reversibility. (medscape.com)
Carnitine2
- Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. (proteopedia.org)
- Crystal structures of murine carnitine acetyltransferase in ternary complexes with its substrates. (proteopedia.org)
Functional1
- A decrease in functional E1 alpha leads to reduced activity of the pyruvate dehydrogenase complex. (medlineplus.gov)
Lactic acid3
- Pyruvate dehydrogenase deficiency is characterized by the buildup of a chemical called lactic acid in the body and a variety of neurological problems. (medlineplus.gov)
- Problems with the breaking down (metabolizing) of pyruvate can limit a cell's ability to produce energy and allow a buildup of a waste product called lactic acid (lactic acidosis). (msdmanuals.com)
- Deficiency results in elevation of pyruvate and thus elevation of lactic acid levels. (merckmanuals.com)