Screened for by all state newborn screening (NBS) programs in the United States, mitochondrial acetoacetyl-coenzyme A thiolase (T2), or ?-ketothiolase, deficiency is a rare autosomal recessive disorder that causes ketoacidosis and hypoglycemia/hyperglycemia. Outcomes vary from normal development to severe cognitive impairment or even ...

The thiolase family is a widespread group of proteins present in prokaryotes and three cellular compartments of eukaryotes. This fact makes this family interesting in order to study the evolutionary process of eukaryotes. Using the sequence of peroxisomal thiolase from Saccharomyces cerevisiae recently obtained by us and the other known ...

Complementary DNAs encoding the precursor of human hepatic mitochondrial acetoacetyl-CoA thiolase (T2) (EC 2.3.1.9) were cloned and sequenced. The cDNA inserts in these clones were 1,518 bases in length when overlapped, and encoded the 427-amino acid precursor of this enzyme (45,199 mol wt). This amino acid sequence included a ...

Thiolases are enzymes that remove an acetyl-coenzyme A group from acyl-CoA in the catabolic ?-oxidation of fatty acids, or catalyse the reverse condensation reaction for anabolic processes such as the biosynthesis of sterols and ketone bodies. In humans, six homologous isoforms of thiolase have been described, differing from each other in sequence, ...

The sorting of homologous proteins between two separate intracellular organelles is a major unsolved problem. 3-Oxoacyl-CoA thiolase is localized in mitochondria and peroxisomes, and provides a good system for the study on the problem. Unlike most mitochondrial matrix proteins, mitochondrial 3-oxoacyl-CoA thiolase in rats is synthesized with no transient ...

Most peroxisomal matrix proteins possess a carboxy-terminal tripeptide targeting signal, termed peroxisomal targeting signal type 1 (PTS1), and follow a relatively well-characterized pathway of import into the organelle. The peroxisomal targeting signal type 2 (PTS2) pathway of peroxisomal matrix protein import is less well understood. In this study, we investigated the mechanisms of PTS2 protein ...

Acetoacetyl-CoA thiolase (AT) is an enzyme that catalyses the CoA-dependent thiolytic cleavage of acetoacetyl-CoA to yield 2 molecules of acetyl-CoA, or the reverse condensation reaction. A full-length cDNA clone pBSGT-3, which has homology to known thiolases, was isolated from Dictyostelium cDNA library. Expression of the protein encoded in pBSGT-3 in ...

The chromosomal location of the human gene coding for peroxisomal 3-oxoacyl-CoA thiolase (ACAA) was determined with the aid of cDNA and genomic probes by screening of rodent x human somatic cell hybrids and in situ hybridization. The results localize the gene to chromosome region 3p22----p23. PMID:2630187

A thl gene encoding the thiolase (EC 2.3.1.9) of Clostridium pasteurianum was cloned by thermal asymmetric interlaced (TAIL) PCR. It consists of 1179 bp with 36.8% GC content and encodes 392 amino acids with a deduced molecular mass of 40,954 Da and shows 77% identity and 88% similarity to that of Clostridium tetani E88 and should be classified as a biosynthetic ...

Thiolase I and II coexist as part of the glyoxysomal ?-oxidation system in sunflower (Helianthus annuus L.) cotyledons, the only system shown to have both forms. The importance of thiolases can be underscored not only by their ubiquity, but also by their involvement in a wide variety of processes in plants, animals and bacteria. Here we describe the ...

The rhizomelic form of chondrodysplasia punctata (RCDP) is a peroxisomal disorder characterized biochemically by an impairment of plasmalogen biosynthesis and phytanate catabolism. We have now found that the maturation of peroxisomal 3-oxoacyl-CoA thiolase is impaired in fibroblasts from RCDP patients. To establish the subcellular localization of the 3-oxoacyl-CoA ...

Mitochondrial acetoacetyl-CoA thiolase deficiency (or beta-ketothiolase deficiency) is a rare metabolic disorder characterized by acute episodes of severe acidosis and ketosis. A case is presented of an 18-month-old boy who presented with vomiting and diarrhoea and was found to be markedly acidotic. When the acidosis persisted despite saline fluid boluses and bicarbonate ...

Contents: General Introduction; Luciferase as a reporter for gene expression studies in yeast; Regulation of transcription of the gene coding for peroxisomal 3-oxoacyl-CoA thiolase in Saccharomyces cerevisiae; A transcriptional control element serves as a...

, Aloe arborescens enzymes are found to be basal to the rest of the family including enzymes from bryophytes, suggesting their appearance during earlier evolution of the family (Fig. 7). A. arborescens

Thiolases are important in fatty-acid degradation and biosynthetic pathways. Analysis of the genomic sequence of Mycobacterium smegmatis suggests the presence of several putative thiolase genes. One of these genes appears to code for an SCP-x protein. Human SCP-x consists of an N-terminal domain (referred to as SCP2 thiolase) and a ...

Thiolase I and II coexist as part of the glyoxysomal beta-oxidation system in sunflower (Helianthus annuus L.) cotyledons, the only system shown to have both forms. The importance of thiolases can be underscored not only by their ubiquity, but also by their involvement in a wide variety of processes in plants, animals and bacteria. Here we describe the ...

Thiolase I and II coexist as part of the glyoxysomal ?-oxidation system in sunflower (Helianthus annuus L.) cotyledons, the only system shown to have both forms. The importance of thiolases can be underscored not only by their ubiquity, but also by their involvement in a wide variety of processes in plants, animals and bacteria. Here we describe the ...

The first step of the butanol pathway involves an acetyl-CoA acetyltransferase (ACoAAT), which controls the key branching point from acetyl-CoA to butanol. ACoAAT, also known as thiolase (EC 2.3.1.9), is encoded by the thl gene and catalyzes ligation of 2 acetyl-CoA into acetoacetyl-CoA. Bioinform...

The first step of the butanol pathway involves an acetyl-CoA acetyltransferase (ACoAAT), which controls the key branching point from acetyl-CoA to butanol. ACoAAT, also known as thiolase (EC 2.3.1.9), is encoded by the thl gene and catalyzes ligation of 2 acetyl-CoA into acetoacetyl-CoA. Bioinform...

t T - t1/3 + t2t1] + P2X4(T: - T2t2). 3. 2. 3. 2. 2. 1 5 2. 3. 2. 3. 2. 2 j3,,. = PlU2[ -T2/3 + T2(t2 + tl /2) - ...

In an attempt to establish why the brain is virtually incapable of oxidizing fatty acids, the activities of the ..beta..-oxidation enzymes in rat brain and rat heart mitochondria were measured and compared with each other. Although the apparent K/sub m/ values and chain-length specificities of the brain and heart enzymes are similar, the specific activities of all but one brain enzyme are between ...

... Title : IN VITRO FORMATION OF 3'-HYDROXY T-2 AND 3'-HYDROXY HT-2 TOXINS FROM T-2 TOXIN BY LIVER HOMOGENATES FROM MICE ...

... Title : GROWTH INHIBITION OF YEAST BY T-2, HT-2, T-2 TRIOL, T-2 TETRAOL, DIACETOXYSCIRPENOL, VERRUCAROL, VERRUCARIN A, AND ...

We investigated the peroxisomal beta-oxidation system in liver from a patient with clinical features similar to those in the cerebrohepatorenal (Zellweger) syndrome and with elevated levels in body fluids of very-long-chain fatty acids and intermediates in the biosynthesis of bile acids. The peroxisomal beta-oxidation of fatty acids, measured as the cyanide-insensitive formation of [14C]acetyl ...

The authors investigated the peroxisomal ..beta..-oxidation system in liver from a patient with clinical features similar to those in the cerebrohepatorenal (Zellweger) syndrome and with elevated levels in body fluids of very-long-chain fatty acids and intermediates in the biosynthesis of bile acids. The peroxisomal ..beta..-oxidation of fatty acids, measured as the cyanide-insensitive formation ...

Thiolase (acetyl coenzyme A acetyltransferase; EC 2.3.1.9) from Clostridium acetobutylicum is a key enzyme in the production of acid and solvents in this organism. The purification and properties of the enzyme have already been described. The thl gene encoding the thiolase has been cloned by using primary antibodies raised to the purified enzyme. A ...

Acetoacetyl-CoA thiolase (EC 2.3.1.9), also called thiolase II, condenses two molecules of acetyl-CoA to give acetoacetyl-CoA. This is the first enzymatic step in the biosynthesis of isoprenoids via mevalonate (MVA). In this work, thiolase II from alfalfa (MsAACT1) was identified and cloned. The enzymatic activity was experimentally ...

The present study deals with toxicological effects and metabolism of the trichothecene mycotoxin T-2 toxin. T-2 toxin was shown to cause a marked decrease in cell production of bone marrow 24 hours after T-2 toxin administration. Furthermore, necrotic inj...

... However, the in vivo effect of Oral T-2 toxin, as measured by leucopenia in actively immunized rats, was not dramatically significant. ...

Phyllostegia hirsuta Viola oahuensis T2 None South Kaukonahua Subunit II Sanicula purpurea T2 None T2 Hesperomannia arborescens T1 Huperzia nutans T1 Viola oahuensis T2 None Reintroductions: MU Name viridiflora ...

The breakdown of fatty acids, performed by the beta-oxidation cycle, is crucial for plant germination and sustainability. beta-Oxidation involves four enzymatic reactions. The final step, in which a two-carbon unit is cleaved from the fatty acid, is performed by a 3-ketoacyl-CoA thiolase (KAT). The shortened fatty acid may then pass through the cycle again (until reaching ...

The breakdown of fatty acids, performed by the ?-oxidation cycle, is crucial for plant germination and sustainability. ?-Oxidation involves four enzymatic reactions. The final step, in which a two-carbon unit is cleaved from the fatty acid, is performed by a 3-ketoacyl-CoA thiolase (KAT). The shortened fatty acid may then pass through the cycle again (until reaching ...

The biosynthesis of the peroxisomal enzymes acyl-CoA oxidase, 3-oxoacyl-CoA thiolase (acetyl-CoA acyl-transferase, EC 2.3.1.16), and catalase (EC 1.11.1.6) was studied in cultured skin fibroblasts from a control subject and from patients with Zellweger syndrome and the infantile form of Refsum disease, inherited disorders in which peroxisomes are deficient and certain ...

Acetoacetyl coenzyme A (acetoacetyl-CoA) thiolase, an enzyme required for short-chain fatty acid degradation, has been purified to near homogeneity from Caulobacter crescentus. The relative heat stability of this enzyme allowed it to be separated from beta-ketoacyl-CoA thiolase. The purification scheme minus the heating step also permitted the ...

... 1.5 4.28 (M) A144T 2.6 3.11 (M) A145T 2.0 3.92 (i) A146T 2.6 6.13 (i) A152T 2.6 6.10 (i). A154T 2.6 5.33 (M) A155T 2.0 ...

, was about 4.4, comparable to the major short-chain thiolase of the butyrate producing Clostridium- morpho1ino)propanesulfonic acid; PAGE, polyacrylamidegel electro- phoresis; PHB, poly-S-CoA) moleculesdifferentially, utilizing one as aC-2- carbanion equivalent and the other as anelectrophile at C-1, to effect

... Table- 1 Analysis-of FE:B-Negative-A.--eutrophus Stain PHB Thiolase' -Reductase 2 Poye e3- ... Red -Too Sma Bgl -co cd Thiol Eco B I Bma-Bar-iHid ...

an exhaustive six-dimensional search. Various definitions of C were suggested (Wriggers and Chaco�n, 2001. A catalase tetramer (PDB entry 7CAT) and one of its monomers were low- pass filtered (Chaco�n and Wriggers Fig. 7. Docking precision tested on four systems: thiolase, PDB entry 1AFW; catalase, PDB entry 7CAT

Advanced (long-chain) fuels and chemicals are generated from short-chain metabolic intermediates through pathways that require carbon-chain elongation. The condensation reactions mediating this carbon-carbon bond formation can be catalysed by enzymes from the thiolase superfamily, including ?-ketoacyl-acyl-carrier protein (ACP) synthases, polyketide synthases, ...

The kinetics of benzoate degradation by the anaerobic syntrophic bacterium, Syntrophus buswellii, was studied in coculture with Desulfovibrio strain G11. The threshold value for benzoate degradation was dependent on the acetate concentration with benzoate threshold values ranging from 2.4 {mu}M at 20 mM acetate to 30.0 {mu}M at 65 mM acetate. Increasing acetate concentrations also inhibited the ...

The kinetics of benzoate degradation by the anaerobic syntrophic bacterium, Syntrophus buswellii, was studied in coculture with Desulfovibrio strain G11. The threshold value for benzoate degradation was dependent on the acetate concentration with benzoate threshold values ranging from 2.4 [mu]M at 20 mM acetate to 30.0 [mu]M at 65 mM acetate. Increasing acetate concentrations also inhibited the ...

Metabolism This collection curated by Stephen Chanock (sc83a@nih.gov) Gene Description Sequences Sequence assembly Predicted SNPs having score >= 0.99 ACAT1 Acetyl-Coenzyme A acetyltransferase 1 (acetoacetyl Coenzyme A thiolase) D90228 D10511 ACAT2 Acetyl-Coenzyme

Peroxisomal 3-ketoacyl-CoA thiolase B (Thb) catalyzes the final step in the peroxisomal ?-oxidation of straight-chain acyl-CoAs and is under the transcription control of the nuclear hormone receptor PPAR?. PPAR? binds to and is activated by the synthetic compound Wy14,643 (Wy). Here, we show that the magnitude of Wy-mediated induction of peroxisomal ?-oxidation of radiolabeled ...

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1-T2-1, Solar Cell, Double Junction GaAs with CP1 coverslide, 3 x 5 cm ... 1-T2- 16, Solar Cell, DSP Silicon with Mylar D coverslide, 3 x 5 cm ...

... A3: Gaussian, � Ordinary Random Walk, Ordinary Brownian Motion, � IEX(t)2 = 2D|t|, Einstein relation, ... IEX(t)2 = 2D|t| γ , � IEX(t)2 = �. ...

Page 1. 1 AF2T2EA � An Illustrative Example of the C2 Conceptual Model by Dr. Paul W. Phister, Jr. ... AF2T2EA An Illustrative Example 22 June 2006 ...

... Abstract : The reported pathophysiologic studies of acute T-2 intoxication reported are ... cellular effects (in addition to direct cellular toxin effects) from ...

Recording Wild T-2 observations at entrance to Chesapeake Bay: Maryland, Chesapeake Bay : 3511 : Level instrument and Wild T-2: 3512 : ... Eastern Gulf of Mexico 1925:

T2.01 Foundational Research for Aeronautics Experimental Capabilities ... identification, and simulation for control objectives are encouraged. ...

T2.01 Aerospace Vehicles Flight Dynamic Modeling and Simulation ... structures, heat transfer, and propulsion disciplines: Emphasis is placed on the ...

... Title : 3'-HYDROXY T-2 AND 3'-HYDROXY HT-2 TOXINS: NEW METABOLITES OF T-2 TOXIN, A TRICHOTHECENE MYCOTOXIN, IN ANIMALS. ...

Jul 21, 2010 ... [T2 snubber arm inspection is no longer needed after every T2 ... time trajectory of the SLAMMD guide arm and a micro controller which ...