5-Aminolevulinate Synthetase: An enzyme of the transferase class that catalyzes condensation of the succinyl group from succinyl coenzyme A with glycine to form delta-aminolevulinate. It is a pyridoxyal phosphate protein and the reaction occurs in mitochondria as the first step of the heme biosynthetic pathway. The enzyme is a key regulatory enzyme in heme biosynthesis. In liver feedback is inhibited by heme. EC 2.3.1.37.Aminolevulinic Acid: A compound produced from succinyl-CoA and GLYCINE as an intermediate in heme synthesis. It is used as a PHOTOCHEMOTHERAPY for actinic KERATOSIS.Porphobilinogen Synthase: An enzyme that catalyzes the formation of porphobilinogen from two molecules of 5-aminolevulinic acid. EC 4.2.1.24.Anemia, Sideroblastic: Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.Glutamate-Ammonia Ligase: An enzyme that catalyzes the conversion of ATP, L-glutamate, and NH3 to ADP, orthophosphate, and L-glutamine. It also acts more slowly on 4-methylene-L-glutamate. (From Enzyme Nomenclature, 1992) EC 6.3.1.2.Amino Acyl-tRNA Synthetases: A subclass of enzymes that aminoacylate AMINO ACID-SPECIFIC TRANSFER RNA with their corresponding AMINO ACIDS.2',5'-Oligoadenylate Synthetase: An enzyme that catalyzes the conversion of ATP into a series of (2'-5') linked oligoadenylates and pyrophosphate in the presence of double-stranded RNA. These oligonucleotides activate an endoribonuclease (RNase L) which cleaves single-stranded RNA. Interferons can act as inducers of these reactions. EC 2.7.7.-.Hydroxymethylbilane Synthase: An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8Heptanoates: Salts and esters of the 7-carbon saturated monocarboxylic acid heptanoic acid.Boranes: The collective name for the boron hydrides, which are analogous to the alkanes and silanes. Numerous boranes are known. Some have high calorific values and are used in high-energy fuels. (From Grant & Hackh's Chemical Dictionary, 5th ed)Hemin: Chloro(7,12-diethenyl-3,8,13,17-tetramethyl-21H,23H-porphine-2,18-dipropanoato(4-)-N(21),N(22),N(23),N(24)) ferrate(2-) dihydrogen.Allylisopropylacetamide: An allylic compound that acts as a suicide inactivator of CYTOCHROME P450 by covalently binding to its heme moiety or surrounding protein.Dicarbethoxydihydrocollidine: 1,4-Dihydro-2,4,6-trimethyl-3,5-pyridinedicarboxylic acid diethyl ester.Amino Acids, Neutral: Amino acids with uncharged R groups or side chains.Rhodobacter capsulatus: Non-pathogenic ovoid to rod-shaped bacteria that are widely distributed and found in fresh water as well as marine and hypersaline habitats.Heme: The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Genetic Diseases, X-Linked: Genetic diseases that are linked to gene mutations on the X CHROMOSOME in humans (X CHROMOSOME, HUMAN) or the X CHROMOSOME in other species. Included here are animal models of human X-linked diseases.PorphobilinogenPeptide Synthases: Ligases that catalyze the joining of adjacent AMINO ACIDS by the formation of carbon-nitrogen bonds between their carboxylic acid groups and amine groups.Keratosis: Any horny growth such as a wart or callus.Kinetics: The rate dynamics in chemical or physical systems.Coenzyme A Ligases: Enzymes that catalyze the formation of acyl-CoA derivatives. EC 6.2.1.Pyridoxal Phosphate: This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).Porphyrins: A group of compounds containing the porphin structure, four pyrrole rings connected by methine bridges in a cyclic configuration to which a variety of side chains are attached. The nature of the side chain is indicated by a prefix, as uroporphyrin, hematoporphyrin, etc. The porphyrins, in combination with iron, form the heme component in biologically significant compounds such as hemoglobin and myoglobin.Schiff Bases: Condensation products of aromatic amines and aldehydes forming azomethines substituted on the N atom, containing the general formula R-N:CHR. (From Grant & Hackh's Chemical Dictionary, 5th ed)Ferrochelatase: A mitochondrial enzyme found in a wide variety of cells and tissues. It is the final enzyme in the 8-enzyme biosynthetic pathway of HEME. Ferrochelatase catalyzes ferrous insertion into protoporphyrin IX to form protoheme or heme. Deficiency in this enzyme results in ERYTHROPOIETIC PROTOPORPHYRIA.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Intramolecular Transferases: Enzymes of the isomerase class that catalyze the transfer of acyl-, phospho-, amino- or other groups from one position within a molecule to another. EC 5.4.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.Pyridoxal: The 4-carboxyaldehyde form of VITAMIN B 6 which is converted to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid.Ligases: A class of enzymes that catalyze the formation of a bond between two substrate molecules, coupled with the hydrolysis of a pyrophosphate bond in ATP or a similar energy donor. (Dorland, 28th ed) EC 6.Erythroblasts: Immature, nucleated ERYTHROCYTES occupying the stage of ERYTHROPOIESIS that follows formation of ERYTHROID PRECURSOR CELLS and precedes formation of RETICULOCYTES. The normal series is called normoblasts. Cells called MEGALOBLASTS are a pathologic series of erythroblasts.Immunologic Tests: Immunologic techniques involved in diagnosis.Aspartate-tRNA Ligase: An enzyme that activates aspartic acid with its specific transfer RNA. EC 6.1.1.12.Methionine-tRNA Ligase: An enzyme that activates methionine with its specific transfer RNA. EC 6.1.1.10.Tryptophan-tRNA Ligase: An enzyme that activates tryptophan with its specific transfer RNA. EC 6.1.1.2.Iron-Regulatory Proteins: Proteins that regulate cellular and organismal iron homeostasis. They play an important biological role by maintaining iron levels that are adequate for metabolic need, but below the toxicity threshold.Carbon-Nitrogen Ligases: Enzymes that catalyze the joining of two molecules by the formation of a carbon-nitrogen bond. EC 6.3.Protoporphyrins: Porphyrins with four methyl, two vinyl, and two propionic acid side chains attached to the pyrrole rings. Protoporphyrin IX occurs in hemoglobin, myoglobin, and most of the cytochromes.Isoleucine-tRNA Ligase: An enzyme that activates isoleucine with its specific transfer RNA. EC 6.1.1.5.Aspartate-Ammonia Ligase: An enzyme that catalyzes the formation of asparagine from ammonia and aspartic acid, in the presence of ATP. EC 6.3.1.1.Glycine: A non-essential amino acid. It is found primarily in gelatin and silk fibroin and used therapeutically as a nutrient. It is also a fast inhibitory neurotransmitter.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Pyridoxine: The 4-methanol form of VITAMIN B 6 which is converted to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. Although pyridoxine and Vitamin B 6 are still frequently used as synonyms, especially by medical researchers, this practice is erroneous and sometimes misleading (EE Snell; Ann NY Acad Sci, vol 585 pg 1, 1990).Phenylalanine-tRNA Ligase: An enzyme that activates phenylalanine with its specific transfer RNA. EC 6.1.1.20.Tyrosine-tRNA Ligase: An enzyme that activates tyrosine with its specific transfer RNA. EC 6.1.1.1.Photochemotherapy: Therapy using oral or topical photosensitizing agents with subsequent exposure to light.Spectrophotometry: The art or process of comparing photometrically the relative intensities of the light in different parts of the spectrum.Carbamoyl-Phosphate Synthase (Ammonia): An enzyme that catalyzes the formation of carbamoyl phosphate from ATP, carbon dioxide, and ammonia. This enzyme is specific for arginine biosynthesis or the urea cycle. Absence or lack of this enzyme may cause CARBAMOYL-PHOSPHATE SYNTHASE I DEFICIENCY DISEASE. EC 6.3.4.16.Circular Dichroism: A change from planar to elliptic polarization when an initially plane-polarized light wave traverses an optically active medium. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Leucine-tRNA Ligase: An enzyme that activates leucine with its specific transfer RNA. EC 6.1.1.4.Serine-tRNA Ligase: An enzyme that activates serine with its specific transfer RNA. EC 6.1.1.11.Valine-tRNA Ligase: An enzyme that activates valine with its specific transfer RNA. EC 6.1.1.9Cloning, Molecular: The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.Argininosuccinate Synthase: An enzyme of the urea cycle that catalyzes the formation of argininosuccinic acid from citrulline and aspartic acid in the presence of ATP. Absence or deficiency of this enzyme causes the metabolic disease CITRULLINEMIA in humans. EC 6.3.4.5.Acetate-CoA Ligase: An enzyme that catalyzes the formation of CoA derivatives from ATP, acetate, and CoA to form AMP, pyrophosphate, and acetyl CoA. It acts also on propionates and acrylates. EC 6.2.1.1.Enzyme Induction: An increase in the rate of synthesis of an enzyme due to the presence of an inducer which acts to derepress the gene responsible for enzyme synthesis.Glutamate-tRNA Ligase: An enzyme that activates glutamic acid with its specific transfer RNA. EC 6.1.1.17.

delta-Aminolevulinate synthetases in the liver cytosol fraction and mitochondria of mice treated with allylisopropylacetamide and 3,5-dicarbethoxyl-1,4-dihydrocollidine. (1/369)

Hepatic delta-aminolevulinate (ALA) synthetase was induced in mice by the administration of allylisopropylacetamide (AIA) and 3,5-dicarbethoxy-1,4-dihydrocollidine (DDC). In both cases, a significant amount of ALA synthetase accumulated in the liver cytosol fraction as well as in the mitochondria. The apparent molecular weight of the cytosol ALA synthetase was estimated to be 320,000 by gel filtration, but when the cytosol ALA synthetase was subjected to sucrose density gradient centrifugation, it showed a molecular weight of 110,000. In the mitochondria, there were two different sizes of ALA synthetase with molecular weights of 150,000 and 110,000, respectively; the larger enzyme was predominant in DDC-treated mice, whereas in AIA-treated mice and normal mice the enzyme existed mostly in the smaller form. When hemin was injected into mice pretreated with DDC, the molecular size of the mitochondrial ALA synthetase changed from 150,000 to 110,000. The half-life of ALA synthetase in the liver cytosol fraction was about 30 min in both the AIA-treated and DDC-treated mice. The half-life of the mitochondrial ALA synthetase in AIA-treated mice and normal mice was about 60 min, but in DDC-treated mice the half-life was as long as 150 min. The data suggest that the cytosol ALA synthetase of mouse liver is a protein complex with properties very similar to those of the cytosol ALA synthetase of rat liver, which has been shown to be composed of the enzyme active protein and two catalytically inactive binding proteins, and that ALA synthetase may be transferred from the liver cytosol fraction to the mitochondria with a size of about 150,000 daltons, followed by its conversion to enzyme with a molecular weight of 110,000 within the mitochondria. The process of intramitochondrial enzyme degradation seems to be affected in DDC-treated animals.  (+info)

Four new mutations in the erythroid-specific 5-aminolevulinate synthase (ALAS2) gene causing X-linked sideroblastic anemia: increased pyridoxine responsiveness after removal of iron overload by phlebotomy and coinheritance of hereditary hemochromatosis. (2/369)

X-linked sideroblastic anemia (XLSA) in four unrelated male probands was caused by missense mutations in the erythroid-specific 5-aminolevulinate synthase gene (ALAS2). All were new mutations: T647C, C1283T, G1395A, and C1406T predicting amino acid substitutions Y199H, R411C, R448Q, and R452C. All probands were clinically pyridoxine-responsive. The mutation Y199H was shown to be the first de novo XLSA mutation and occurred in a gamete of the proband's maternal grandfather. There was a significantly higher frequency of coinheritance of the hereditary hemochromatosis (HH) HFE mutant allele C282Y in 18 unrelated XLSA hemizygotes than found in the normal population, indicating a role for coinheritance of HFE alleles in the expression of this disorder. One proband (Y199H) with severe and early iron loading coinherited HH as a C282Y homozygote. The clinical and hematologic histories of two XLSA probands suggest that iron overload suppresses pyridoxine responsiveness. Notably, reversal of the iron overload in the Y199H proband by phlebotomy resulted in higher hemoglobin concentrations during pyridoxine supplementation. The proband with the R452C mutation was symptom-free on occasional phlebotomy and daily pyridoxine. These studies indicate the value of combined phlebotomy and pyridoxine supplementation in the management of XLSA probands in order to prevent a downward spiral of iron toxicity and refractory anemia.  (+info)

Properties of 5-aminolaevulinate synthetase and its relationship to microsomal mixed-function oxidation in the southern armyworm (Spodoptera eridania). (3/369)

1. Activity of 5-aminolaevulinate synthetase was measured in the midgut and other tissues of the last larval instar of the southern armyworm (Spodoptera eridania Cramer, formerly Prodenia eridania Cramer). 2. Optimum conditions for measuring the activity were established with respect to all variables involved and considerable differences from those reported for mammalian enzyme preparations were found. 3. Maximum activity (20 nmol/h per mg of protein) occurs 18-24 h after the fifth moult and thereafter decreases to trace amounts as the larvae age and approach pupation. 4. Synthetase activity was rapidly induced by oral administration (in the diet) of pentamethylbenzene, phenobarbital, diethyl 1,4-dihydro-2,4,6-trimethylpyridine-3, 5-dicarboxylate, and 2-allyl-2-isopropylacetamide. 5. Puromycin inhibited the induction of synthetase by pentamethylbenzene. 6. Induction of 5-aminolaevulinate synthetase correlated well with the induction of microsomal N-demethylation of p-chloro-N-methylaniline, except for phenobarbital, which induced the microsomal oxidase relatively more than the synthetase.  (+info)

Pre-steady-state reaction of 5-aminolevulinate synthase. Evidence for a rate-determining product release. (4/369)

5-Aminolevulinate synthase (ALAS) is the first enzyme of the heme biosynthetic pathway in non-plant eukaryotes and the alpha-subclass of purple bacteria. The pyridoxal 5'-phosphate cofactor at the active site undergoes changes in absorptive properties during substrate binding and catalysis that have allowed us to study the kinetics of these reactions spectroscopically. Rapid scanning stopped-flow experiments of murine erythroid 5-aminolevulinate synthase demonstrate that reaction with glycine plus succinyl-CoA results in a pre-steady-state burst of quinonoid intermediate formation. Thus, a step following binding of substrates and initial quinonoid intermediate formation is rate-determining. The steady-state spectrum of the enzyme is similar to that formed in the presence of 5-aminolevulinate, suggesting that release of this product limits the overall rate. Reaction of either glycine or 5-aminolevulinate with ALAS is slow (kf = 0.15 s-1) and approximates kcat. The rate constant for reaction with glycine is increased at least 90-fold in the presence of succinyl-CoA and most likely represents a slow conformational change of the enzyme that is accelerated by succinyl-CoA. The slow rate of reaction of 5-aminolevulinate with ALAS is 5-aminolevulinate-independent, suggesting that it also represents a slow isomerization of the enzyme. Reaction of succinyl-CoA with the enzyme-glycine complex to form a quinonoid intermediate is a biphasic process and may be irreversible. Taken together, the data suggest that turnover is limited by release of 5-aminolevulinate or a conformational change associated with 5-aminolevulinate release.  (+info)

Phylogenetic analysis of the 5-aminolevulinate synthase gene. (5/369)

The evolution of 5-aminolevulinate synthase (ALS) was studied by acquiring sequence data and generating phylogenetic trees. Gene sequences were already available for a variety of vertebrates (which have both a housekeeping and an erythroid form of the gene), fungi, alpha-proteobacteria, and one protist and one protostome. In order to generate representative trees, ALS sequence data were acquired from various deuterostomes and protostomes. The species and tissues selected for study were beluga whale liver, hagfish blood, sea urchin gonadal tissue, cuttlefish hepatopancreas, horseshoe crab hepatopancreas, and bloodworm blood. The new sequences and those previously published were examined for the presence of heme-regulatory motifs (HRMs) and iron-responsive elements (IREs). The HRMs are present in almost all eukaryotic species, which suggests their fundamental role in the regulation of ALS. The IREs are present in all vertebrate erythroid forms of ALS, which indicates that in those animals, expression of the erythroid form of the enzyme and, hence, hemoglobin production can be influenced by the intracellular content of iron. The new sequences were aligned with previously reported ALS sequences, and phylogenetic analyses were performed. The resulting trees provided evidence regarding the timing of the gene duplication event that led to the two forms of the ALS gene in vertebrates. It appears that the housekeeping and erythroid forms of ALS probably arose before the divergence of hagfish from the deuterostome line leading to the vertebrates. The data also add to the evidence indicating that alpha-proteobacteria are the nearest contemporary relatives of mitochondria.  (+info)

Respiratory uncoupling induces delta-aminolevulinate synthase expression through a nuclear respiratory factor-1-dependent mechanism in HeLa cells. (6/369)

Nuclear respiratory factor (NRF)-1 appears to be important for the expression of several respiratory genes, but there is no direct evidence that NRF-1 transduces a physiological signal into the production of an enzyme critical for mitochondrial biogenesis. We generated HeLa cells containing plasmids allowing doxycycline-inducible expression of uncoupling protein (UCP)-1. In the absence of doxycycline, UCP-1 mRNA and protein were undetectable. In the presence of doxycycline, UCP-1 was expressed and oxygen consumption doubled. This rise in oxygen consumption was associated with an increase in NRF-1 mRNA. It was also associated with an increase in NRF-1 protein binding activity as determined by electrophoretic mobility shift assay using a functional NRF-1 binding site from the delta-aminolevulinate (ALA) synthase promoter. Respiratory uncoupling also caused a time-dependent increase in protein levels of ALA synthase, an early marker for mitochondrial biogenesis. ALA synthase induction by respiratory uncoupling was prevented by transfecting cells with an oligonucleotide antisense to the region of the NRF-1 initiation codon; a scrambled oligonucleotide with the same base composition had no effect. Respiratory uncoupling increases oxygen consumption and lowers energy reserves. In HeLa cells, uncoupling also increases ALA synthase, an enzyme critical for mitochondrial respiration, but only if translatable mRNA for NRF-1 is available. These data suggest that the transcription factor NRF-1 plays a key role in cellular adaptation to energy demands by translating physiological signals into an increased capacity for generating energy.  (+info)

Characterization of the rhodobacter sphaeroides 5-aminolaevulinic acid synthase isoenzymes, HemA and HemT, isolated from recombinant Escherichia coli. (7/369)

The hemA and hemT genes encoding 5-aminolaevulinic acid synthase (ALAS) from the photosynthetic bacterium Rhodobacter sphaeroides, were cloned to allow high expression in Escherichia coli. Both HemA and HemT appeared to be active in vivo as plasmids carrying the respective genes complemented an E. coli hemA strain (glutamyl-tRNA reductase deficient). The over-expressed isoenzymes were isolated and purified to homogeneity. Isolated HemA was soluble and catalytically active whereas HemT was largely insoluble and failed to show any activity ex vivo. Pure HemA was recovered in yields of 5-7 mg x L-1 of starting bacterial culture and pure HemT at 10 mg x L-1 x HemA has a final specific activity of 13 U x mg-1 with 1 unit defined as 1 micromol of 5-aminolaevulinic acid formed per hour at 37 degrees C. The Km values for HemA are 1.9 mM for glycine and 17 microM for succinyl-CoA, with the enzyme showing a turnover number of 430 h-1. In common with other ALASs the recombinant R. sphaeroides HemA requires pyridoxal 5'-phosphate (PLP) as a cofactor for catalysis. Removal of this cofactor resulted in inactive apo-ALAS. Similarly, reduction of the HemA-PLP complex using sodium borohydride led to > 90% inactivation of the enzyme. Ultraviolet-visible spectroscopy with HemA suggested the presence of an aldimine linkage between the enzyme and pyridoxal 5'-phosphate that was not observed when HemT was incubated with the cofactor. HemA was found to be sensitive to reagents that modify histidine, arginine and cysteine amino acid residues and the enzyme was also highly sensitive to tryptic cleavage between Arg151 and Ser152 in the presence or absence of PLP and substrates. Antibodies were raised to both HemA and HemT but the respective antisera were not only found to bind both enzymes but also to cross-react with mouse ALAS, indicating that all of the proteins have conserved epitopes.  (+info)

A photosensitising adenovirus for photodynamic therapy. (8/369)

We have developed a new approach to photodynamic therapy based on adenoviral transduction of the rate-limiting enzyme in heme synthesis. Conventional phototherapy uses porphyrin-based chemical photosensitisers, including delta-aminolaevulinic acid (ALA) which is converted to protoporphyrin IX (PpIX) by the enzymes of the heme biosynthetic pathway. The lack of a specific mechanism for targeting chemical photosensitisers and PpIX to tumour cells means that therapeutic irradiation can damage normal tissue and exposure to sunlight following treatment can cause severe burns. The rate limiting enzyme in PpIX synthesis is ALA-synthase (ALA-S). We have developed a new yeast vector system for manipulation of the adeno- virus genome and used it to construct a virus expressing a mutant form of ALA-S lacking the iron response elements which regulate ALA-S translation and the heme regulatory motifs which regulate import of ALA-S into mitochondria. The virus induces a large increase in PpIX expression and confers photosensitivity on cultured cells. Unlike conventional photodynamic therapy, a viral approach makes it possible to restrict photosensitivity by biological rather than purely physical or chemical means. As with HSV thymidine kinase, ALA-S expression is a general mechanism for sensitisation to a therapeutic agent which can easily be adapted to whatever means of gene delivery is most effective.  (+info)

Endotoxin was administered to rats at a dose shown previously to stimulate hepatic haem oxygenase activity and to block induction of delta-aminolaevulinate synthase, apparently by causing redistribution of haem from cytochrome P-450 to a regulatory haem pool in the liver. Within 5h of the administration of endotoxin (at a time when the effect of the compound on cytochrome P-450 is maximal) the relative saturation of tryptophan pyrrolase with intrinsic haem rose, from a basal value of 50% to 90%, indicating that free haem had become available. Concurrently, the activity of delta-aminolaevulinate synthase was decreased to 25% of its basal value. Haem oxygenase reached peak activity 13h after endotoxin administration. These findings provide new evidence for the existence of an unassigned hepatic haem fraction, which exchanges with cytochrome P-450 haem and regulates these three enzyme functions. ...
TY - JOUR. T1 - Induction of hepatic δ-aminolevulinate synthase and cytochrome P-450 by a thiazide diuretic. AU - Anderson, K. E.. AU - Kappas, A.. PY - 1981/1/1. Y1 - 1981/1/1. UR - http://www.scopus.com/inward/record.url?scp=17144448883&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=17144448883&partnerID=8YFLogxK. M3 - Article. AN - SCOPUS:17144448883. VL - 1. SP - 1A. JO - Hepatology. JF - Hepatology. SN - 0270-9139. IS - 5. ER - ...
Methyl aminolevulinate photodynamic therapy, Methyl aminolevulinate PDT, Metvix PDT, MAL PDT. Authoritative facts from DermNet New Zealand.
Methyl aminolevulinate makes your skin more sensitive to light. It works by causing a reaction with light that can destroy certain types of diseased skin cells.
Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:. ...
How is X-Linked Sideroblastic Anemia abbreviated? XLSA stands for X-Linked Sideroblastic Anemia. XLSA is defined as X-Linked Sideroblastic Anemia somewhat frequently.
Congenital Sideroblastic Anemia (CSA) is a group of rare inherited disorders that decrease the number of red blood cells. Learn more from Boston Childrens Hospital
The activity of 5-aminolaevulinate (ALA) synthase, the first and rate-limiting of haem synthesis, was markedly reduced (13% of controls) in erythroblasts of a patient with acquired, primary sideroblastic anaemia (PASA). The reduced activity of ALA synthase could not be restored in vitro with 1 mmol/l pyridoxal-5-phosphate (PLP). Treatment of the patient with pyridoxine for several months increased the ALA synthase activity from 13% to 50% of controls in the absence and to 100% in the presence of PLP in the incubation medium. These studies suggest that both increased degradation of apo-ALA synthase and decreased affinity of ALA synthase for PLP may be involved in pyridoxine-responsive PASA ...
Aminolevulinic acid synthase (ALA synthase, ALAS, or delta-aminolevulinic acid synthase) is an enzyme (EC 2.3.1.37) that catalyzes the synthesis of D-aminolevulinic acid (ALA) the first common precursor in the biosynthesis of all tetrapyrroles such as hemes, cobalamins and chlorophylls. The reaction is as follows: succinyl-CoA + glycine ⇌ {\displaystyle \rightleftharpoons } δ-aminolevulinic acid + CoA + CO2 This enzyme is expressed in all non-plant eukaryotes and the α-class of proteobacteria. Other organisms produce ALA through a three enzyme pathway known as the Shemin pathway. ALA is synthesized through the condensation of glycine and succinyl-CoA. In humans, transcription of ALA synthase is tightly controlled by the presence of Fe2+-binding elements, to prevent accumulation of porphyrin intermediates in the absence of iron. There are two forms of ALA synthase in the body. One form is expressed in red blood cell precursor cells (ALAS2), whereas the other (ALAS1) is ubiquitously expressed ...
Cultured chick embryo hepatocytes were iron-loaded with ferric nitrilotriacetate. Iron-loading was confirmed by both quantitative cellular iron determinations and ultrastructural studies. With iron-loading, lipid peroxidation, as detected by malonaldehyde released into the medium, occurred at a linear rate for 12h, after which time the rate of malonaldehyde production decreased. No cell toxicity, as detected by lactate dehydrogenase release, was noted. The amount of malonaldehyde recovered in the medium after 18h of exposure to iron represented 24-33% of the total malonaldehyde that could be produced by incubating lysed cells with iron and ascorbate. Cellular glutathione was not affected by iron-stimulated lipid peroxidation, but was increased by allylisopropylacetamide. Although iron-loading by itself had no effect on activity of 5-aminolaevulinate synthase, the first and rate-limiting step in haem synthesis, iron-loading in the presence of the porphyrogenic drug allylisopropylacetamide ...
A 3-month-old boy presented with decreased appetite, fatigue, and a nosebleed. Initial workup revealed hemoglobin 2.6 g/dL (10.2-12.7), hematocrit 7.7% (30.9-37.9), mean corpuscular volume 104 fL (71.3-82.6), white blood cell count 4200/μL (240 absolute neutrophil count), platelets 50 000/μL (140-400), and reticulocyte count 1.31% (1.55-2.7). Bone marrow revealed hypercellularity with cytoplasmic vacuolization of myeloid and erythroid precursors (panels A-B; original magnification ×1000, Wright-Giemsa stain). Iron staining of marrow biopsy revealed numerous ringed sideroblasts (panels C-D; original magnification ×1000, Prussian blue stain). DNA was examined by array-based comparative genomic hybridization and revealed a 4.9-kb deletion, m.11027_15950del4923, consistent with mitochondrial DNA deletion syndrome, also known as Pearson syndrome. Fecal elastase was initially normal, but now it is ,15 μg/g (normal ,200), consistent with severe pancreatic insufficiency. We have begun pancreatic ...
A decade ago, Brooks McMurrays routine check-up was anything but routine. The suburban Boston boys spleen was enlarged. His red blood cell count was low and the cells were very small and very pale, which suggested a serious iron deficiency anemia. The family pediatrician referred McMurray, now a 19-year-old college freshman, to Dana-Farber/Boston Childrens Cancer and Blood Disorders Center.. There hematologists discovered the boy had unexpectedly high iron levels. Together with pathologist Mark Fleming, MD, DPhil, they solved the mystery. McMurray has congenital sideroblastic anemia, an inherited blood disorder so rare that fewer than 1,000 cases have been reported worldwide. Iron was getting stuck in the wrong place in the precursor red blood cells developing in his bone marrow. …. ...
Find the best sideroblastic anemia doctors in Kolkata. Get guidance from medical experts to select sideroblastic anemia specialist in Kolkata from trusted hospitals - credihealth.com
The product of this gene specifies an erythroid-specific mitochondrially located enzyme. The encoded protein catalyzes the first step in the heme biosynthetic pathway. Defects in this gene cause X-linked pyridoxine-responsive sideroblastic anemia. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008 ...
Metvix (methyl aminolevulinate) (MAL), Photodynamic Therapy (PDT), cream, 160 mg/g. MAL cream will be applied for 3 hours then will be removed. The target area will then be exposed to red light (using a large-field LED light source: Aktilite 128) for 7 to 10 minutes at a dosage of 37 J/cm² ...
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Glutamate-1-semialdehyde-2,1-aminomutase (GSAM) catalyzes the isomerization of glutamate-1-semialdehyde (GSA) to 5-aminolevulinate (ALA) and is distributed in archaea, most bacteria and plants. of DAVA (Fig. 1 ?, step 2 2). The intermediate DAVA is definitely then produced accompanied by the formation of an internal aldimine between PLP and the active-site lysine part chain (Fig. 1 ?, step… Read more Glutamate-1-semialdehyde-2,1-aminomutase (GSAM) catalyzes the isomerization of glutamate-1-semialdehyde (GSA) to 5-aminolevulinate (ALA). ...
Learn about the causes, symptoms, diagnosis & treatment of Anemias Caused by Deficient Erythropoiesis from the Professional Version of the Merck Manuals.
ANEMIA, SIDEROBLASTIC, 3, PYRIDOXINE-REFRACTORY; SIDBA3 description, symptoms and related genes. Get the complete information in our medical search en
X-linked sideroblastic anemia or "X-linked dominant erythropoietic protoporphyria", associated with ALAS2 (aminolevulinic acid synthase), has also been described. X-linked dominant erythropoietic protoporphyria (XDEPP) is caused by a gain of function mutation in the ALAS2 (5-aminolevulinate synthase) gene; that gene encodes the very first enzyme in the heme biosynthetic pathway. The mutation is caused by a frameshift mutation caused by one of two deletions in the ALAS2 exon 11, either c. 1706-1709 delAGTG or c. 1699-1700 delAT. This alters the 19th and 20th residues of the C-terminal domain thereby altering the secondary structure of the enzyme. The delAT mutation only occurred in one family studied whereas the delAGTG mutation occurred in several genetically distinct families. The delAGTG causes a loss of an α-helix which is replaced by a β-sheet. Previously known mutations in the ALAS2 resulted in a loss-of-function mutation causing X-linked sideroblastic anemia. Erythropoietic ...
YARS2 variants have previously been described in patients with myopathy, lactic acidosis and sideroblastic anemia 2 (MLASA2). YARS2 encodes the mitochondrial tyrosyl-tRNA synthetase, which is responsible for conjugating tyrosine to its cognate mt-tRNA for mitochondrial protein synthesis. Here we describe 14 individuals from 11 families presenting with sideroblastic anemia and YARS2 variants that we identified using a sideroblastic anemia gene panel or exome sequencing. The phenotype of these patients ranged from MLASA to isolated congenital sideroblastic anemia. As in previous cases, inter- and intra-familial phenotypic variability was observed, however, this report includes the first cases with isolated sideroblastic anemia and patients with biallelic YARS2 variants that have no clinically ascertainable phenotype. We identified ten novel YARS2 variants and three previously reported variants. In vitro amino-acylation assays of five novel missense variants showed that three had less effect on the ...
Looking for online definition of idiopathic sideroblastic anemia in the Medical Dictionary? idiopathic sideroblastic anemia explanation free. What is idiopathic sideroblastic anemia? Meaning of idiopathic sideroblastic anemia medical term. What does idiopathic sideroblastic anemia mean?
... Primary Hereditary Sideroblastic Anemia. Primary Acquired Refractory Anemia With Ringed Sideroblasts (RARS). Sideroblastic Anemia Information Including: BASIC INFORMATION, SIGNS AND SYMPTOMS, EPIDEMIOLOGY & DEMOGRAPHICS, PHYSICAL FINDINGS & CLINICAL PRESENTATION, LABORATORY TESTS. DIAGNOSIS, TREATMENT and more
At least 50 mutations that cause X-linked sideroblastic anemia have been identified in the ALAS2 gene. Almost all of these mutations change single protein building blocks (amino acids) in erythroid ALA-synthase. These changes impair the activity of the enzyme, which disrupts the normal production of heme in developing red blood cells. A reduction in the amount of heme prevents these cells from making enough hemoglobin. Because almost all of the iron transported into erythroblasts is normally incorporated into heme, the reduced production of heme leads to a buildup of excess iron in these cells. Additionally, the body attempts to compensate for the hemoglobin shortage by absorbing more iron from the diet. This buildup of excess iron can damage the bodys organs. Low hemoglobin levels and the resulting accumulation of iron in the bodys organs lead to the characteristic features of X-linked sideroblastic anemia. ...
The essential features of X-linked sideroblastic anemia include the following: (1) a hypochromic microcytic anemia and 2 discrete populations of red blood cells, one microcytic and the other normocytic; (2) marrow ringed sideroblasts, particularly prominent in the late erythroid precursors; (3) a variable hematologic response to pharmacologic doses of pyridoxine; and (4) systemic iron overload secondary to chronic ineffective erythropoiesis. The age of clinical onset of the disorder can vary from in utero to the ninth decade. Whereas males are preferentially affected, females may present with clinically severe anemia. More commonly, female carriers of the disease have an increased red blood cell distribution width and sometimes erythrocyte dimorphism ({18:Fleming, 2002 ...
Lauwerys, R. R., Buchet, J.-P., and Roels, H. A. (1973).British Journal of Industrial Medicine,30, 359-364. Comparative study of effect of inorganic lead and cadmium on blood δ-aminolevulinate dehydratase in man. δ-Aminolevulinate dehydratase (ALA1-D) of red blood cells, lead concentration in blood (Pb-B) and in urine (Pb-U), cadmium concentration in blood (Cd-B) and in urine (Cd-U), and ALA in urine (ALA-U) were measured in 77 workers occupationally exposed to cadmium, and in 73 control workers.. An excellent negative correlation was found between log ALA-D and Pb-B (r = - 0·660) or Pb-U (r = - 0·501), but no significant correlation was found between Cd-B and log ALA-D activity.. Unlike ALA-D, ALA-U is not correlated with Pb and Pb-U in the `normal range of Pb concentration investigated. Mean ALA-D activity in smokers is lower than in nonsmokers, and this is probably related to the fact that a higher mean Pb-B concentration is found in smokers than in nonsmokers.. It is clear from this ...
The latest electronic edition of the journal Nature Genetics reports the discovery of a new gene responsible for congenital sideroblastic anemia.
Diagnosis Code D64.0 information, including descriptions, synonyms, code edits, diagnostic related groups, ICD-9 conversion and references to the diseases index.
MetabolismBiosynthesis of cofactors, prosthetic groups, and carriersHeme, porphyrin, and cobalamin5-aminolevulinic acid synthase (TIGR01821; EC 2.3.1.37; HMM-score: 184.5) ...
Anemia, Sideroblastic answers are found in the 5-Minute Clinical Consult powered by Unbound Medicine. Available for iPhone, iPad, Android, and Web.
"Sideroblastic anemia" . Os 7 Tipos de Anemia e Seus Principais Sintomas. estrutural hereditário dos glóbulos vermelhos (Anemia de células falciformes), ou uma incapacidade para realizar ou utilizar a hemoglobina (Anemia sideroblástica). Então, confira Os 7 Tipos de Anemia
BACKGROUND: Pearson syndrome is a rare mitochondrial disorder characterized by sideroblastic anemia, liver disease, renal tubulopathy and exocrine pancreas deficiency. OBSERVATIONS: We describe a female infant suffering from anemia since birth who gr
Study Flashcards On FA Biochemistry Heme Synthesis at Cram.com. Quickly memorize the terms, phrases and much more. Cram.com makes it easy to get the grade you want!
Looking for online definition of delta-aminolevulinate dehydratase porphyria in the Medical Dictionary? delta-aminolevulinate dehydratase porphyria explanation free. What is delta-aminolevulinate dehydratase porphyria? Meaning of delta-aminolevulinate dehydratase porphyria medical term. What does delta-aminolevulinate dehydratase porphyria mean?
Alzheimers disease (AD) is a neurodegenerative pathology that affects elderly people all over the world. Several studies have demonstrated that oxidative stress is an aggravating factor for AD development and progression. Therefore, this study aimed to evaluate the activity of two oxidative stress markers, glutathione peroxidase (GPx) and δ -aminolevulinate dehydratase ( δ -ALA-D), as well as correlate them with blood metal levels and AD progression. For this purpose, 88 elderly individuals were divided in two groups: AD group (34 patients diagnosed with AD) and control group (34 subjects paired by age with the AD group). The Mini-Mental State Examination and the Clinical Dementia Rating (CDR ) were used as tools to classify the AD progression. GPx and δ -ALA-D activities were measured in all subjects through blood tests. Both enzymes activities were decreased in AD patients when compared to the age-matched control group, regardless of the CDR. Moreover, GPx activity was positively ...
Modified tetrapyrroles are versatile compounds that are universally utilized by enzymes as co-factors in a myriad of enzyme cata lyzed reaction s. Examples include heme, the cofactor of hemoglobin and myoglobin, that carries and stores molecular oxygen in the blood and muscle respectively, and cobalamin a cofactor that is synthesized by prokaryotes and which is an important nutrient for humans. This work focuses on the enzymatic mechanism of metal chelation and in particular on the structural characterization of the cobalt-chelatases CbiK from Salmonella enterica and CbiX from Archaeoglobus /u/gidus that take part in the biosynthesis of cobalamin. Crystal structures of the enzyme-tetrapyrrole complex were obtained that reveal radically different modes of binding compared to the well characterized ferrochelatases. Furthermore protein structures reveal the evolutionary re lationships between cobaltochelatases from different organisms. The second part of this thesis is focusing on 5-aminolevulinic ...
Macrocytic Anemia in Manifesting Females Symptom Checker: Possible causes include Hereditary Sideroblastic Anemia & Macrocytic Anemia & Sideroblastic Anemia. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.
Subcellular Localization of Iron and Heme Metabolism Related Proteins at Early Stages of Erythrophagocytosis. . Biblioteca virtual para leer y descargar libros, documentos, trabajos y tesis universitarias en PDF. Material universiario, documentación y tareas realizadas por universitarios en nuestra biblioteca. Para descargar gratis y para leer online.
Lookup HS Codes for Taiwan vi 28.13.90 Commerical phosphorus trisulphide. Avalara LandedCosts helps determine your duty rates and other import taxes for Taiwan.
Biological Sciences Shirley, Cat.No. AD00876Z Description Adenovirus with ORF of aminolevulinate dehydratase (ALAD) with C terminal Flag and His tag....
Basal cell carcinoma (BCC) is the most common cancer affecting Caucasians and, due to its large size or to the poor condition of the patient, it can be difficult to treat it with conventional therapies: in these cases photodynamic therapy with methyl aminolevulinate (MAL-PDT) may represent a good option. A retrospective non-comparative follow-up study was performed to test the response of giant and large BCC to MAL-PDT. Twelve patients with 14 giant BCC (≥ 5 cm) and 5 patients with 5 large BCC (4-5 cm) were treated with MAL-PDT; they were evaluated 6 months after the end of the treatment to define the initial cure rate, and then at 12 and 36 months for the follow-up. At 6 months the initial cure rate for the 19 BCCs was 95% and at 36 months the overall long-term cure rate was 66%. The follow-up will last up to 5 years. MAL-PDT is a valid option for the treatment of giant and large BCC ...
Semantic Scholar extracted view of Determination of delta-aminolevulinic acid in blood plasma and urine by gas-liquid chromatography. by J. MacGee et al.
Differential Effects of Mitomycin C on Constitutive and Inducible Gene Expression in the Chicken Embryo Liver In Vivo: Correlation with Developmental Age and Chromatin Structure A Thesis Submitted to the Faculty in partial fulfillment of the requirements for the degree of Doctor of Philosophy in Pharmacology and Toxicology by Rosemary M. Caron DARTMOUTH COLLEGE Hanover, New Hampshire October 13, 1995 ...
Six patients had primary sideroblastic erythropoiesis together with a haemoglobin concentration of 12.0 g/dl or higher. In four cases this was associated with macrocytosis. Other abnormalities included failure of erythroid progenitor growth from peripheral blood in three cases and occasional dysplastic appearances in neutrophils and megakaryocytes. Sideroblastic erythropoiesis seems to be an early manifestation of the myelodysplastic syndrome and may present clinically at a pre-anaemic stage.. ...
The pathophysiology of myelodysplasia is complex. There is evidence for impairments in stem cell growth, progenitor maturation, and both growth factor production and progenitor responsiveness. The presence of ineffective hematopoiesis (increased apoptosis of maturing marrow precursors) is a hallmark of myelodysplasia. It appears to correlate in part with CD95 expression and persistent high levels of Fas receptor, resulting in increased fas-ligand apoptosis. Upregulation of the cytokines tumor necrosis factor α (TNF-α) and interleukin 1β (IL-1β) also may play a role in promoting apoptosis of long-term hematopoietic stem cells (LT-HSC) and committed precursors in myelodysplastic marrows. Vascular endothelial growth factor (VEGF) overproduction is thought to be involved in the promotion of myeloblastic elements, and perhaps the evolution to acute myeloid leukemia (AML). ...
The pathophysiology of myelodysplasia is complex. There is evidence for impairments in stem cell growth, progenitor maturation, and both growth factor production and progenitor responsiveness. The presence of ineffective hematopoiesis (increased apoptosis of maturing marrow precursors) is a hallmark of myelodysplasia. It appears to correlate in part with CD95 expression and persistent high levels of Fas receptor, resulting in increased fas-ligand apoptosis. Upregulation of the cytokines tumor necrosis factor α (TNF-α) and interleukin 1β (IL-1β) also may play a role in promoting apoptosis of long-term hematopoietic stem cells (LT-HSC) and committed precursors in myelodysplastic marrows. Vascular endothelial growth factor (VEGF) overproduction is thought to be involved in the promotion of myeloblastic elements, and perhaps the evolution to acute myeloid leukemia (AML). ...
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We recently determined that erythropoietin (EPO) activates 3 members of the signal transducer and activator of transcription (STAT) family, Stat1α, Stat3, and Stat5, in the human EPO-dependent cell lines, UT-7 and UT-7/EPO (Kirito et al, J Biol Chem 272:16507, 1997). In addition, we have shown that Stat1α, but not Stat3, is involved in EPO-induced cellular proliferation. In this study, we examined the roles of Stat1α and Stat3 in EPO-induced erythroid differentiation. UT-7/GM was used as a model system, because this cell line can differentiate into erythroid-lineage cells with EPO treatment (Komatsu et al, Blood 89:4021, 1997). We found that EPO did not activate Stat1α or Stat3 in UT-7/GM cells. Transfection experiments showed that both Stat1α and Stat3 inhibited the induction by EPO of γ-globin and erythroid-specific 5-aminolevulinate synthetase transcripts, resulting in a reduction of the percentage of hemoglobin-positive cells. Dominant negative forms of Stat1α or Stat3 promoted the ...
Givosiran is synthetic small interfering RNA (siRNA) molecule directed against 5-aminolevulinic acid synthase that is used to treat acute hepatic porphyria. Givosiran has been linked to mild-to-moderate ALT elevations during therapy, but has not been linked to instances of idiosyncratic acute liver injury with symptoms and jaundice.
... succinyl-CoA synthetase, and mitoferrin-1. Multiple studies have suggested the existence of an oligomeric complex that enables ... 269 (1): 390-5. PMID 8276824. Wang X, Poh-Fitzpatrick M, Carriero D, Ostasiewicz L, Chen T, Taketani S, Piomelli S (April 1993 ... 89 (1): 281-5. doi:10.1073/pnas.89.1.281. PMC 48220 . PMID 1729699. Lamoril J, Boulechfar S, de Verneuil H, Grandchamp B, ... 378 (5): 1074-1083. doi:10.1016/j.jmb.2008.03.040. PMC 2852141 . PMID 18423489. Bencze, Krisztina Z.; Yoon, Taejin; Mill?n- ...
... amino acyl-trna synthetases MeSH D08.811.464.263.200.050 --- alanine-tRNA ligase MeSH D08.811.464.263.200.100 --- arginine-tRNA ... atp synthetase complexes MeSH D08.811.913.696.650.150.500 --- proton-translocating atpases MeSH D08.811.913.696.650.150.500.249 ... fatty acid synthetase complex MeSH D08.811.600.391 --- glycine decarboxylase complex MeSH D08.811.600.391.100 --- ... uroporphyrinogen iii synthetase MeSH D08.811.520.241.700 --- polysaccharide-lyases MeSH D08.811.520.241.700.350 --- ...
Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha ... Succinyl-CoA synthetase SUCLG1 SUCLG2 GRCh38: Ensembl release 89: ENSG00000136143 - Ensembl, May 2017 GRCm38: Ensembl release ... Furuyama K, Sassa S (March 2000). "Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is ... Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), also known as ADP-forming succinyl-CoA synthetase (SCS- ...
... argininosuccinate synthetase BNC2: zinc finger protein basonuclin-2 C9orf64: chromosome 9 open reading frame 64 C9orf78: ... aminolevulinate, delta-, dehydratase ALS4: amyotrophic lateral sclerosis 4 ANGPTL2: angiopoietin-related protein 2 ASS: ... 5 (2): 157-74. doi:10.1089/109065701753145664. PMID 11551106. Humphray SJ, Oliver K, Hunt AR, et al. (2004). "DNA sequence and ... 11 (5): 206. doi:10.1186/gb-2010-11-5-206. PMC 2898077 . PMID 20441615. "Statistics & Downloads for chromosome 9". HUGO Gene ...
LARS2: leucyl-tRNA synthetase, mitochondrial. *LIMD1: LIM domain-containing protein 1. *LINC00312: Long intergenic non-protein- ... ALAS1: aminolevulinate, delta-, synthase 1. *APEH: encoding enzyme Acylamino-acid-releasing enzyme ... doi:10.1186/gb-2010-11-5-206. PMC 2898077. PMID 20441615.. *^ "Statistics & Downloads for chromosome 3". HUGO Gene Nomenclature ... So CCDS's gene number prediction represents a lower bound on the total number of human protein-coding genes.[5] ...
Delta-aminolevulinate synthase 2 also known as ALAS2 is a protein that in humans is encoded by the ALAS2 gene. ALAS2 is an ... Furuyama K, Sassa S (Mar 2000). "Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is ... "Entrez Gene: Delta-aminolevulinate synthase 2". Human ALAS2 genome location and ALAS2 gene details page in the UCSC Genome ... Cotter PD, Willard HF, Gorski JL, Bishop DF (May 1992). "Assignment of human erythroid delta-aminolevulinate synthase (ALAS2) ...
Partial list of the genes located on p-arm (short arm) of human chromosome 3: ALAS1: aminolevulinate, delta-, synthase 1 APEH: ... leucyl-tRNA synthetase, mitochondrial LIMD1: LIM domain-containing protein 1 LINC00312: Long intergenic non-protein-coding RNA ... 11 (5): 206. doi:10.1186/gb-2010-11-5-206. PMC 2898077 . PMID 20441615. "Statistics & Downloads for chromosome 3". HUGO Gene ... ETS variant 5 FAM43A: family with sequence similarity 43 member A FAM162A: family with sequence similarity 162 member A GYG1: ...
Additionally, alcohol has been shown to increase the activity of the delta-aminolevulinic acid synthetase (ALA synthetase), the ... 14 (38): 5913-5. doi:10.3748/wjg.14.5913. PMC 2751904 . PMID 18855993. Sökmen, M; Demirsoy, H; Ersoy, O; Gökdemir, G; Akbayir, ... 18 (3): 200-5. PMID 17891697. Frank, J; Poblete-Gutiérrez, P; Weiskirchen, R; Gressner, O; Merk, H. F.; Lammert, F (2006). " ... 58 (5): 1089-97. doi:10.1172/JCI108560. PMC 333275 . PMID 993332. Di Padova, C.; Marchesi, L.; Cainelli, T.; Gori, G.; ...
AKs are one of the most common dermatologic lesions for which photodynamic therapy using topical methyl aminolevulinate (MAL) ... destroys AKs by blocking methylation of thymidylate synthetase, thereby interrupting DNA and RNA synthesis. This in turn ... Topical 5-FU is the most utilized treatment for AK, and often results in effective removal of the lesion. Overall, there is a ... 5-FU may be up to 90% effective in treating non-hyperkeratotic lesions. The most commonly used application regimen consists of ...
There are two different asparagine synthetases found in bacterial species. These two synthetases, which are both referred to as ... Porphyrins are synthesized from glycine and succinyl CoA, which condense to give δ-aminolevulinate. Two molecules of this ... When there is too much of any one of them, that one will allosterically control the DAHP synthetase by "turning it off". With ... The conversion of glutamate to glutamine is regulated by glutamine synthetase (GS) and is a highly significant step in nitrogen ...
... which is made of both nonribosomal peptide synthetase (NRPS) and polyketide synthase (PKS) modules. Nonribosomal peptides and ... Pingyangmycin (Bleomycin A5). References[edit]. *^ a b c d e f g h i j k l m n o p q r "Bleomycin Sulfate". The American ... 3-{[(2'-{(5S,8S,9S,10R,13S)-15-{6-amino-2- [(1S)-3-amino-1-{[(2S)-2,3-diamino-3-oxopropyl]amino}-3-oxopropyl] -5- ... 5: 13419. doi:10.1038/srep13419. PMC 4542162. PMID 26289670. Archived from the original on 2016-05-03. In our studies, mice ...
Looking for Erythroid 5-aminolevulinate synthetase deficiency? Find out information about Erythroid 5-aminolevulinate ... synthetase deficiency. A usually hereditary, pathologic disorder of porphyrin metabolism characterized by porphyrinuria and ... The little-known disease porphyria is... Explanation of Erythroid 5-aminolevulinate synthetase deficiency ... redirected from Erythroid 5-aminolevulinate synthetase deficiency). Also found in: Dictionary, Thesaurus, Medical. porphyria. [ ...
67417 Ears2; glutamyl-tRNA synthetase 2, mitochondrial 107508 Eprs; glutamyl-prolyl-tRNA synthetase 17025 Alad; aminolevulinate ... glutamyl-tRNA synthetase [EC:6.1.1.17] K14163 EPRS; bifunctional glutamyl/prolyl-tRNA synthetase [EC:6.1.1.17 6.1.1.15] K01698 ... 15159 Hccs; holocytochrome c synthetase 12870 Cp; ceruloplasmin 15203 Heph; hephaestin 14297 Fxn; frataxin K00643 E2.3.1.37; 5- ... 22247 Umps; uridine monophosphate synthetase 17960 Nat1; N-acetyl transferase 1 17961 Nat2; N-acetyltransferase 2 (arylamine N- ...
... was used to unravel the diversity and phylogeny of genes encoding 5-aminolevulinic acid synthases (ALASs, hemA gene products) ... was used to unravel the diversity and phylogeny of genes encoding 5-aminolevulinic acid synthases (ALASs, hemA gene products) ... Mayer, S. M., and Beale, S. I. (1992). Succinyl-coenzyme A synthetase and its role in delta-aminolevulinic acid biosynthesis in ... This specifically concerns hemA alleles encoding cyclizing type of aminolevulinate synthase, as a part of the C5N-encoding gene ...
... succinyl-CoA synthetase, and mitoferrin-1. Multiple studies have suggested the existence of an oligomeric complex that enables ... 269 (1): 390-5. PMID 8276824. Wang X, Poh-Fitzpatrick M, Carriero D, Ostasiewicz L, Chen T, Taketani S, Piomelli S (April 1993 ... 89 (1): 281-5. doi:10.1073/pnas.89.1.281. PMC 48220 . PMID 1729699. Lamoril J, Boulechfar S, de Verneuil H, Grandchamp B, ... 378 (5): 1074-1083. doi:10.1016/j.jmb.2008.03.040. PMC 2852141 . PMID 18423489. Bencze, Krisztina Z.; Yoon, Taejin; Mill?n- ...
... amino acyl-trna synthetases MeSH D08.811.464.263.200.050 --- alanine-tRNA ligase MeSH D08.811.464.263.200.100 --- arginine-tRNA ... atp synthetase complexes MeSH D08.811.913.696.650.150.500 --- proton-translocating atpases MeSH D08.811.913.696.650.150.500.249 ... fatty acid synthetase complex MeSH D08.811.600.391 --- glycine decarboxylase complex MeSH D08.811.600.391.100 --- ... uroporphyrinogen iii synthetase MeSH D08.811.520.241.700 --- polysaccharide-lyases MeSH D08.811.520.241.700.350 --- ...
A transcription factor that controls the expression of variety of proteins including CYTOCHROME C and 5-AMINOLEVULINATE ... SYNTHETASE. It plays an important role in maintenance of the RESPIRATORY CHAIN of MITOCHONDRIA. ...
Differential effects of metalloporphyrins on messenger RNA levels of delta-aminolevulinate synthase and heme oxygenase. Studies ... "Differential effects of metalloporphyrins on messenger RNA levels of delta-aminolevulinate synthase and heme oxygenase. Studies ... 5-Aminolevulinate Synthetase; Animals; Cells, Cultured; Chick Embryo; Heme Oxygenase (Decyclizing); Liver; Metalloporphyrins; ...
5-Aminolevulinate Synthetase; Animals; Cells, Cultured; Chick Embryo; Cytochrome P-450 Enzyme System; Deferoxamine; Heme; Liver ...
Chlorophyll Biosynthesis: Various Chlorophyllides as Exogenous Substrates for Chlorophyll Synthetase. Benz, Jürgen / Rüdiger, ... 5-year IMPACT FACTOR: 0.912. CiteScore 2017: 0.92. SCImago Journal Rank (SJR) 2017: 0.288. Source Normalized Impact per Paper ( ... Darstellung 5′.5″-phosphatverknüpfter Dinucleoside / Preparation of 5′,5″-Phosphate Linked Dinucleosides. Bomemann, Siegmar / ... An Evolutionary Tree Based on Monoclonal Antibody-Recognized Surface Features of a Plastid Enzyme (5-Aminolevulinate ...
aminolevulinate, delta-, synthetase 2. 0.813. nkx2.2a. NK2 transcription factor related 2a. 0.812. ...
Association between δ-aminolevulinate dehydratase G177C polymorphism and blood lead levels in brain tumor patients. Taha MM, ... Influence of the common human delta-aminolevulinate dehydratase polymorphism on lead body burden. Wetmur JG. Wetmur JG. Environ ... 2015 Sep;3(5):995-1000. doi: 10.3892/mco.2015.589. Epub 2015 Jun 25. Mol Clin Oncol. 2015. PMID: 26623039 Free PMC article. ... 5 μg/dL). We also tested whether the ALAD genotype modified the relationship between blood lead level and mortality. ...
We show that the rate-limiting enzyme in hepatic heme biosynthesis, 5-aminolevulinate synthase (ALAS-1), is regulated by the ...
IMSEAR is the collaborative product of Health Literature, Library and Information Services (HELLIS) Network Member Libraries in the WHO South-East Asia Region ...
amide synthetase. hypothetical protein, partial, Streptomyces sp. NRRL WC-3742, WP_078910860 ... BiosynthesisC5NPolyketide synthaseRubromycin Streptomyces Introduction. Hyaluromycin is a hyaluronidase inhibitor isolated from ... 5]. The strain grow well on ISP 3, ISP 4 and yeast-starch agars but poor on ISP 2, ISP 5, ISP 6, ISP 7, glucose-asparagine, ... The detected menaquinones were identified as MK-9(H8), MK-9(H6), MK-9(H4) and MK-9(H10) (5:37:57:1). The principal polar lipids ...
... delta-ALA synthetase , aminolevulinate, delta, synthase 1 , delta-ALA synthetase 1 , ALA-synthase , aminolevulinate, delta-, ... anti-Aminoacyl tRNA Synthetase Complex-Interacting Multifunctional Protein 2 Antikörper * anti-Aminoacyl tRNA Synthetase ... delta-aminolevulinate synthase 1 , migration-inducing protein 4 , aminolevulinate synthase H , succinyl-CoA: glycine C-succinyl ... Show all anti-Aminolevulinate, delta-, Synthase 1 (ALAS1) Antikörper with Pubmed References. * Human Polyclonal ALAS1 Primary ...
Aminolevulinate dehydratase deficiency porphyria Synonyms: 5-Aminolevulinic acid dehydratase deficiency porphyria, ALA ... Synonyms: Erythrohepatic protoporphyria, EPP, Heme synthetase deficiency, Ferrochelatase deficiency Hepatoerythropoietic ...
Delta-aminolevulinate dehydratase deficiency porphyria see ALA dehydratase deficiency Dementia see CADASIL ... Heme synthetase deficiency see erythropoietic protoporphyria Hemochromatoses see hemochromatosis hemochromatosis hemoglobin M ... Erythroid 5-aminolevulinate synthetase deficiency see X-linked sideroblastic anemia Erythropoietic porphyria see congenital ... 5-ALA dehydratase-deficient porphyria see ALA dehydratase deficiency 5-aminolaevulinic dehydratase deficiency porphyria see ALA ...
2010, 5: e9181-10.1371/journal.pone.0009181.PubMedPubMed CentralView ArticleGoogle Scholar. ... 2004, 5: 123-135. 10.1038/nrg1271.View ArticleGoogle Scholar. *. Minorsky PV: The hot and the classic. Plant Physiol. 2003, 132 ... 10.1016/S1055-7903(03)00194-5.PubMedView ArticleGoogle Scholar. *. Timmis JN, Ayliffe MA, Huang CY, Martin W: Endosymbiotic ... 10.1016/S0168-9525(00)02209-5.PubMedView ArticleGoogle Scholar. *. Martin W: Mosaic bacterial chromosomes - a challenge en ...
aminolevulinate, delta-, synthetase 2. 0.062. capns1a. calpain, small subunit 1 a. 0.062. ... hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase. 0.023. ... tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein, theta polypeptide b. 0.064. ...
Looking for online definition of erythrogenic in the Medical Dictionary? erythrogenic explanation free. What is erythrogenic? Meaning of erythrogenic medical term. What does erythrogenic mean?
5-Aminolevulinate synthase catalysis: The catcher in heme biosynthesis. Mol Genet Metab. 2019 11; 128(3):178-189. PMID: ... Molecular dynamics analysis of the structural and dynamic properties of the functionally enhanced hepta-variant of mouse 5- ... aminolevulinate synthase. J Biomol Struct Dyn. 2018 01; 36(1):152-165. PMID: 27928941. ... the Dynamics of the Active Site Loop and C-Terminal Tail in Relation to the Homodimer Asymmetry of the Mouse Erythroid 5- ...
holocarboxylase synthetase products*ADM2 products*adrenomedullin products*Hrs products*Prnp products*Vash1 products ... Molecular identification and characterization of two medium-chain acyl-CoA synthetases, MACS1 and the Sa gene product. J Biol ... A Kruppel-like factor KLF15 contributes fasting-induced transcriptional activation of mitochondrial acetyl-CoA synthetase gene ... 1990;5:166-71 pubmed ..m2, and if the PVR is greater than 4 units.m2 with the oxygen inhalation test or 7 units.m2 with the ...
These data indicate that the Cyp2a-4/5 complex is regulated in a different way in DBA/2 and C57BL/6 mice and that some ... 5 aminolevulinate synthetase*nuclease protection assays*steroid 16 alpha hydroxylase*beta naphthoflavone*ferrochelatase* ... Salonpää P, Iscan M, Pasanen M, Arvela P, Pelkonen O, Raunio H. Cerium-induced strain-dependent increase in Cyp2a-4/5 ( ... Effect of pyrazole, cobalt and phenobarbital on mouse liver cytochrome P-450 2a-4/5 (Cyp2a-4/5) expression. Biochem J. 1992;286 ...
Glutathione synthetase. Glycine amidinotransferase, mitochondrial. Serine--pyruvate aminotransferase. Serine ... The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the bodys production of DNA, phospholipids and ... The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the bodys production of DNA, phospholipids and ... 5-aminolevulinate synthase, nonspecific, mitochondrial. Glycine receptor subunit alpha-1. Glycine receptor subunit alpha-2. ...
Aminolevulinate dehydratase deficiency porphyria Synonyms: 5-Aminolevulinic acid dehydratase deficiency porphyria, ALA ... Synonyms: Erythrohepatic protoporphyria, EPP, Heme synthetase deficiency, Ferrochelatase deficiency Erythropoietic uroporphyria ...
  • The 5-aminolevulinic acid dehydratase (ALAD) G177C genetic polymorphism (rs 1800435) affects lead toxicokinetics and may alter the adverse effects of lead exposure. (cdc.gov)
  • Comprehensive analysis of 5-aminolevulinic acid dehydrogenase (ALAD) variants and renal cell carcinoma risk among individuals exposed to lead. (cdc.gov)
  • A combined approach, comprising PCR screening and genome mining, was used to unravel the diversity and phylogeny of genes encoding 5-aminolevulinic acid synthases (ALASs, hemA gene products) in streptomycetes-related strains. (frontiersin.org)
  • We consider the presence of this gene triad to be a specific genetic marker of the C 5 N unit biosynthetic pathway that "tags" producers of C 5 N-containing metabolites among others. (frontiersin.org)
  • The principal aim of our work was to show the potential of the hemA -targeted genetic screening in identification of novel putative biosynthetic gene clusters encoding C 5 N-containing metabolites. (frontiersin.org)
  • 1 In addition to these well-established functions as a prosthetic group, recent reports have revealed that heme regulates several transcription factors, including Bach1, 2 ⇓ ⇓ - 5 NPAS2, 6 and REV-ERBs, 7 and modulates gene expression as an inter- and intracellular signaling molecule in mammals. (bloodjournal.org)
  • The expression of calcitonin gene-related peptide (CGRP) in the spinal dorsal horn (L2-3 and L4-5) was examined to evaluate central sensitization. (bvsalud.org)
  • In actinomycetes, these genes were believed to be directly connected with the production of secondary metabolites carrying the C 5 N unit, 2-amino-3-hydroxycyclopent-2-enone, with biological activities making them attractive for future use in medicine and agriculture. (frontiersin.org)
  • First, we identified the genes that were differentially expressed in GBMs (3 datasets) compared to non-GBM brain tissues (5 datasets), or were associated with survival differences. (beds.ac.uk)
  • A glutamine synthetase inhibitor, MSX, stimulated ammonium excretion and H 2 -production and pulses of MSX rather than a single dose favoured both the processes. (go.jp)
  • A transcription factor that controls the expression of variety of proteins including CYTOCHROME C and 5-AMINOLEVULINATE SYNTHETASE. (umassmed.edu)
  • 1 The binding of EPO to its specific receptor on the cell surface induces tyrosine phosphorylation and the activation of several proteins, including Janus kinase 2 (JAK2), 2 signal transducer and activator of transcription 5 (Stat5), 3-5 mitogen-associated protein kinases, 6 and phospholipase C-γ1. (ashpublications.org)
  • 5-aminolevulinate (ALA) is a key precursor of a huge family of essential tetrapyrrole compounds. (frontiersin.org)
  • The biosynthetic steps from the earliest universal precursor, 5-aminolevulinic acid (ALA), to protoporphyrin IX-based hemes constitute the major, common portion of the pathway, and other steps leading to specific groups of products can be considered branches off the main axis. (asmscience.org)
  • An SJ, Park SK, Hwang IK, et al (2004) Vigabatrin inhibits pyridoxine-5′-phosphate oxidase, not pyridoxal kinase in the hippocampus of seizure prone gerbils. (springer.com)
  • From early studies of kindreds with EPP, most investigators concluded that the disease displayed autosomal dominant inheritance with low penetrance and an increased propensity to develop chronic liver disease, 5 though autosomal recessive forms of EPP have also been described. (porphyriafoundation.org)
  • It interferes with DNA synthesis by blocking methylation of deoxyuridylic acid and inhibiting thymidylate synthetase and, subsequently, cell proliferation. (medscape.com)
  • The basal activity of ALA synthetase is substantially lower than that of subsequent enzymes in the synthetic pathway, and therefore changes in ALA synthetase activity are rate limiting, controlling the rate of heme synthesis. (medpdfarticles.com)
  • Topical 5% imiquimod is approved by the US Food and Drug Administration (FDA) for the treatment of nonfacial superficial BCCs that are less than 2 cm in diameter. (medscape.com)
  • Total 14 patients had acute myeloid leukemia, 10 had acute lymphoblastic leukemia, 5 had myelodysplastic syndrome, 3 had non-Hodgkin's lymphoma, and 2 had aplastic anemia. (bvsalud.org)
  • These data indicate that the Cyp2a-4/5 complex is regulated in a different way in DBA/2 and C57BL/6 mice and that some association exists between the development of liver damage and COH induction. (labome.org)
  • The structure consists of a γ-rubromycin core possessing a C 5 N unit as an amide substituent of the carboxyl functionality. (biomedcentral.com)
  • GSS (glutathione synthetase) was not differentially expressed, but higher levels were linked to poor progression free survival. (beds.ac.uk)
  • Groups of 2-5 cats ingesting fruit juice containing 730-2000 ppm (0.073-0.2%) tin (3.65-20 mg tin/kg bw) did not excrete any tin in their urine (Benoy et al. (inchem.org)
  • The most common chemotherapeutic agent used in superficial basal cell carcinoma is topical 5-fluorouracil. (medscape.com)