AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesInformation Science
5-Aminolevulinate SynthetaseAminolevulinic AcidPorphobilinogen SynthaseAnemia, SideroblasticGlutamate-Ammonia LigaseAmino Acyl-tRNA Synthetases2',5'-Oligoadenylate SynthetaseHydroxymethylbilane SynthaseHeptanoatesBoranesHeminAllylisopropylacetamideDicarbethoxydihydrocollidineAmino Acids, NeutralRhodobacter capsulatusHemeMolecular Sequence DataGenetic Diseases, X-LinkedPorphobilinogenPeptide SynthasesKeratosisKineticsCoenzyme A LigasesPyridoxal PhosphatePorphyrinsSchiff BasesFerrochelataseBase SequenceIntramolecular TransferasesAmino Acid SequenceEscherichia coliPyridoxalLigasesErythroblastsImmunologic TestsAspartate-tRNA LigaseMethionine-tRNA LigaseTryptophan-tRNA LigaseIron-Regulatory ProteinsCarbon-Nitrogen LigasesProtoporphyrinsIsoleucine-tRNA LigaseAspartate-Ammonia LigaseGlycineRNA, MessengerPyridoxinePhenylalanine-tRNA LigaseTyrosine-tRNA LigasePhotochemotherapySpectrophotometryCarbamoyl-Phosphate Synthase (Ammonia)Circular DichroismLeucine-tRNA LigaseSerine-tRNA LigaseValine-tRNA LigaseCloning, MolecularArgininosuccinate SynthaseAcetate-CoA LigaseEnzyme InductionGlutamate-tRNA Ligase
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 4.3.1.8
Heptanoates
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.
Porphobilinogen
Peptide 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 6.1.1.9
Cloning, 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)

Effect of endotoxin on tryptophan pyrrolase and delta-aminolaevulinate synthase: evidence for an endogenous regulatory haem...Effect of endotoxin on tryptophan pyrrolase and delta-aminolaevulinate synthase: evidence for an endogenous regulatory haem...
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. ...
http://www.biochemj.org/content/166/2/301
Induction of hepatic δ-aminolevulinate synthase and cytochrome P-450 by a thiazide diuretic<...Induction of hepatic δ-aminolevulinate synthase and cytochrome P-450 by a thiazide diuretic<...
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 - ...
https://researchexperts.utmb.edu/en/publications/induction-of-hepatic-%CE%B4-aminolevulinate-synthase-and-cytochrome-p-
Methyl aminolevulinate photodynamic therapy | DermNet NZMethyl aminolevulinate photodynamic therapy | DermNet NZ
Methyl aminolevulinate photodynamic therapy, Methyl aminolevulinate PDT, Metvix PDT, MAL PDT. Authoritative facts from DermNet New Zealand.
https://www.dermnetnz.org/topics/methyl-aminolevulinate-photodynamic-therapy
Methyl Aminolevulinate Topical - Side Effects, Dosage, Interactions - Drugs - Everyday HealthMethyl Aminolevulinate Topical - Side Effects, Dosage, Interactions - Drugs - Everyday Health
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.
https://www.everydayhealth.com/drugs/methyl-aminolevulinate-topical
Methyl Aminolevulinate (Topical Application Route) Side Effects - Mayo ClinicMethyl Aminolevulinate (Topical Application Route) Side Effects - Mayo Clinic
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:. ...
https://www.mayoclinic.org/drugs-supplements/methyl-aminolevulinate-topical-application-route/side-effects/drg-20072169
XLSA - X-Linked Sideroblastic Anemia | AcronymFinderXLSA - X-Linked Sideroblastic Anemia | AcronymFinder
How is X-Linked Sideroblastic Anemia abbreviated? XLSA stands for X-Linked Sideroblastic Anemia. XLSA is defined as X-Linked Sideroblastic Anemia somewhat frequently.
https://www.acronymfinder.com/X_Linked-Sideroblastic-Anemia-
Congenital Sideroblastic Anemia | Boston Childrens HospitalCongenital Sideroblastic Anemia | Boston Childrens Hospital
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
http://www.childrenshospital.org/conditions-and-treatments/conditions/c/congenital-sideroblastic-anemia
Pyridoxine-responsive primary acquired sideroblastic anaemia : in vitro and in vivo effects of vitamin B6 on decreased 5...Pyridoxine-responsive primary acquired sideroblastic anaemia : in vitro and in vivo effects of vitamin B6 on decreased 5...
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 ...
http://edoc.unibas.ch/7780/
Aminolevulinic acid synthase - WikipediaAminolevulinic acid synthase - Wikipedia
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 ...
https://en.wikipedia.org/wiki/Aminolevulinic_acid_synthase
Iron loading of cultured hepatocytes. Effect of iron on 5-aminolaevulinate synthase is independent of lipid peroxidation |...Iron loading of cultured hepatocytes. Effect of iron on 5-aminolaevulinate synthase is independent of lipid peroxidation |...
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 ...
http://www.biochemj.org/content/212/2/321
An infant with Pearson syndrome: a rare cause of congenital sideroblastic anemia and bone marrow failure | Blood JournalAn infant with Pearson syndrome: a rare cause of congenital sideroblastic anemia and bone marrow failure | Blood Journal
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 ...
http://www.bloodjournal.org/content/129/19/2710?sso-checked=true
sideroblastic anemiasideroblastic anemia
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. …. ...
https://vector.childrenshospital.org/tag/sideroblastic-anemia/
Best Sideroblastic Anemia Doctor in Kolkata, Sideroblastic Anemia Doctors | CredihealthBest Sideroblastic Anemia Doctor in Kolkata, Sideroblastic Anemia Doctors | Credihealth
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
https://www.credihealth.com/doctors/kolkata/sideroblastic-anemia
5-aminolevulinate synthase, erythroid-specific, mitochondrial5-aminolevulinate synthase, erythroid-specific, mitochondrial
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 ...
https://pharos.nih.gov/idg/targets/P22557
Compassionate Use of Metvix® (Methyl Aminolevulinate) Photodynamic Therapy (PDT) in Subjects With Field Actinic Keratoses,...Compassionate Use of Metvix® (Methyl Aminolevulinate) Photodynamic Therapy (PDT) in Subjects With Field Actinic Keratoses,...
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 ...
https://clinicaltrials.gov/ct2/show/NCT00535080?cond=%22Bowens+disease%22&rank=3
Human Aminolevulinate Delta Dehydratase (ALAD) ELISA Kit, Cat#EKU02314 - BiomatikHuman Aminolevulinate Delta Dehydratase (ALAD) ELISA Kit, Cat#EKU02314 - Biomatik
You may return most new, unopened items within 30 days of delivery for a full refund. Well also pay the return shipping costs if the return is a result of our error (you received an incorrect or defective item, etc.).. You should expect to receive your refund within four weeks of giving your package to the return shipper, however, in many cases you will receive a refund more quickly. This time period includes the transit time for us to receive your return from the shipper (5 to 10 business days), the time it takes us to process your return once we receive it (3 to 5 business days), and the time it takes your bank to process our refund request (5 to 10 business days).. If you need to return an item, simply login to your account, view the order using the Complete Orders link under the My Account menu and click the Return Item(s) button. Well notify you via e-mail of your refund once weve received and processed the returned item.. ...
https://www.biomatik.com/elisa-kits/human-aminolevulinate-delta-dehydratase-alad-elisa-kit-cat-eku02314/
Long-term sucrose and glucose consumption decreases the δ-aminolevulinate dehydratase activity in miceLong-term sucrose and glucose consumption decreases the δ-aminolevulinate dehydratase activity in mice
A free platform for explaining your research in plain language, and managing how you communicate around it - so you can understand how best to increase its impact.
https://www.growkudos.com/publications/10.1016%252Fj.nut.2007.08.002/reader
August 2017 - Small-Molecule Inhibitors of Protein InteractionsAugust 2017 - Small-Molecule Inhibitors of Protein Interactions
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). ...
http://www.conferencecallsworld.com/2017/08/
Sideroblastic Anemias - Hematology and Oncology - Merck Manuals Professional EditionSideroblastic Anemias - Hematology and Oncology - Merck Manuals Professional Edition
ANEMIA, SIDEROBLASTIC, 3, PYRIDOXINE-REFRACTORY; SIDBA3 | MENDELIAN.COANEMIA, SIDEROBLASTIC, 3, PYRIDOXINE-REFRACTORY; SIDBA3 | MENDELIAN.CO
ANEMIA, SIDEROBLASTIC, 3, PYRIDOXINE-REFRACTORY; SIDBA3 description, symptoms and related genes. Get the complete information in our medical search en
https://www.mendelian.co/diseases/anemia-sideroblastic-3-pyridoxine-refractory-sidba3
Erythropoietic porphyria - WikipediaErythropoietic porphyria - Wikipedia
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 ...
https://en.wikipedia.org/wiki/Erythropoietic_porphyria
The phenotypic spectrum of germline YARS2 variants: from isolated sideroblastic anemia to mitochondrial myopathy, lactic...The phenotypic spectrum of germline YARS2 variants: from isolated sideroblastic anemia to mitochondrial myopathy, lactic...
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 ...
https://repositori.upf.edu/handle/10230/37193
Idiopathic sideroblastic anemia | definition of idiopathic sideroblastic anemia by Medical dictionaryIdiopathic sideroblastic anemia | definition of idiopathic sideroblastic anemia by Medical dictionary
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?
https://medical-dictionary.thefreedictionary.com/idiopathic+sideroblastic+anemia
Sideroblastic Anemia. Basic Information, Causes, Diagnosis and Treatment of Sideroblastic AnemiaSideroblastic Anemia. Basic Information, Causes, Diagnosis and Treatment of Sideroblastic Anemia
Sideroblastic Anemia. Basic Information, Causes, Diagnosis and Treatment of Sideroblastic Anemia. 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
http://encyclopedia.lubopitko-bg.com/Sideroblastic_anemia.html
ALAS2 gene - Genetics Home Reference - NIHALAS2 gene - Genetics Home Reference - NIH
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. ...
https://ghr.nlm.nih.gov/gene/ALAS2
Hereditary sideroblastic anemiaHereditary 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 ...
http://diseaseinfosearch.org/Hereditary+sideroblastic+anemia/8535
Comparative study of effect of inorganic lead and cadmium on blood δ-aminolevulinate dehydratase in man | Occupational &...Comparative study of effect of inorganic lead and cadmium on blood δ-aminolevulinate dehydratase in man | Occupational &...
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 ...
https://oem.bmj.com/content/30/4/359
Gene Responsible For A Rare Form Of Congenital Anemia IdentifiedGene Responsible For A Rare Form Of Congenital Anemia Identified
The latest electronic edition of the journal Nature Genetics reports the discovery of a new gene responsible for congenital sideroblastic anemia.
http://www.medindia.net/news/Gene-Responsible-For-A-Rare-Form-Of-Congenital-Anemia-Identified-51123-1.htm
JCI - Mutations in the iron-sulfur cluster biogenesis protein HSCB cause congenital sideroblastic anemiaJCI - Mutations in the iron-sulfur cluster biogenesis protein HSCB cause congenital sideroblastic anemia
Sequencing. Except as noted, DNA was prepared from peripheral blood using the PureGene DNA isolation kit (Gentra). For Sanger sequencing, the HSCB promoter region (c.-250 to c.-1), exons, and intron-exon junctions were amplified and sequenced using the primers described in Supplemental Table 1 and analyzed with Sequencher 5.4.1 (Gene Codes Corp.). Paired-end whole exome sequencing (WES) libraries were prepared with the Agilent SureSelect V5 exon selection kit and sequenced to a median depth of ~100× with 100 bp paired-end reads on an Illumina NextSeq 2500 instrument. Data were processed on an internally validated pipeline and analyzed on the WuXi NextCODE genomics platform (www.wuxinextcode.com).. Cell culture. Primary fibroblasts and HeLa cells were cultured in DMEM (11965-092, Gibco) and K562 cells in RPMI 1640 medium (11875-093, Gibco). All media were supplemented with 10% FBS (MilliporeSigma), 2 mM glutamine (Corning), and 100 IU/mL penicillin/100 μg/mL streptomycin (Corning) and described ...
https://www.jci.org/articles/view/135479
Anemia, sideroblastic, pyridoxine-refractory autosomal recessive | definition of anemia, sideroblastic, pyridoxine-refractory...Anemia, sideroblastic, pyridoxine-refractory autosomal recessive | definition of anemia, sideroblastic, pyridoxine-refractory...
Looking for online definition of anemia, sideroblastic, pyridoxine-refractory autosomal recessive in the Medical Dictionary? anemia, sideroblastic, pyridoxine-refractory autosomal recessive explanation free. What is anemia, sideroblastic, pyridoxine-refractory autosomal recessive? Meaning of anemia, sideroblastic, pyridoxine-refractory autosomal recessive medical term. What does anemia, sideroblastic, pyridoxine-refractory autosomal recessive mean?
https://medical-dictionary.thefreedictionary.com/anemia%2C+sideroblastic%2C+pyridoxine-refractory+autosomal+recessive
ICD-10 Diagnosis Code D64.0 Hereditary sideroblastic anemiaICD-10 Diagnosis Code D64.0 Hereditary sideroblastic anemia
Diagnosis Code D64.0 information, including descriptions, synonyms, code edits, diagnostic related groups, ICD-9 conversion and references to the diseases index.
https://icdlist.com/icd-10/d640
SACOL RS12725 - AureoWikiSACOL RS12725 - AureoWiki
MetabolismBiosynthesis of cofactors, prosthetic groups, and carriersHeme, porphyrin, and cobalamin5-aminolevulinic acid synthase (TIGR01821; EC 2.3.1.37; HMM-score: 184.5) ...
http://aureowiki.med.uni-greifswald.de/SACOL_RS12725
Anemia, Sideroblastic | 5-Minute Clinical ConsultAnemia, Sideroblastic | 5-Minute Clinical Consult
Anemia, Sideroblastic answers are found in the 5-Minute Clinical Consult powered by Unbound Medicine. Available for iPhone, iPad, Android, and Web.
https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/116028/all/Anemia__Sideroblastic?refer=true
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Sideroblastic anemia -...Sideroblastic anemia -...
"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
http://en.natvim.com/search?q=Sideroblastic+anemia
Pearson syndrome in an infant heterozygous for C282Y allele of HFE gene.Pearson syndrome in an infant heterozygous for C282Y allele of HFE gene.
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
http://www.biomedsearch.com/nih/Pearson-syndrome-in-infant-heterozygous/17852457.html
FA Biochemistry Heme Synthesis Flashcards - Cram.comFA Biochemistry Heme Synthesis Flashcards - Cram.com
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!
http://www.cram.com/flashcards/fa-biochemistry-heme-synthesis-360466
5-Aminolevulinate dehydratase porphyria: Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to...5-Aminolevulinate dehydratase porphyria: Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to...
TY - JOUR. T1 - 5-Aminolevulinate dehydratase porphyria. T2 - Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to hemin. AU - Lahiji, Arian Pourmehdi. AU - Anderson, Karl E.. AU - Chan, Amy. AU - Simon, Amy. AU - Desnick, Robert J.. AU - Ramanujam, V. M.Sadagopa. N1 - Funding Information: This research was supported in part by the Porphyrias Consortium (U54DK083909), which is a part of the NCATS Rare Diseases Clinical Research Network, which is an initiative of the Office of Rare Diseases Research (ORDR), National Center for Advancing Translational Sciences (NCATS), funded through a collaboration between NCATS and the NIDDK, and also with the support of the Institute for Translational Sciences at the University of Texas Medical Branch, supported in part by a Clinical and Translational Science Award (2UL1TR001439-06) from NCATS, National Institutes of Health: and by the American Porphyria Foundation. Funding Information: This research was supported in part by the ...
https://researchexperts.utmb.edu/en/publications/5-aminolevulinate-dehydratase-porphyria-update-on-hepatic-5-amino
Eldorado: Δ-Aminolevulinate dehydratase and glutathione peroxidase activity in Alzheimers diseaseEldorado: Δ-Aminolevulinate dehydratase and glutathione peroxidase activity in Alzheimers disease
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 ...
https://eldorado.tu-dortmund.de/handle/2003/39054
ReP USP - Detalhe do registro: Delta-aminolevulinic acid induced autoxidation of hemoglobinReP USP - Detalhe do registro: Delta-aminolevulinic acid induced autoxidation of hemoglobin
MONTEIRO, H P; ABDALLA, Dulcinéia Saes Parra; BECHARA, Etelvino José Henriques. Delta-aminolevulinic acid induced autoxidation of hemoglobin. Anais.. Roma: [s.n.], 1985 ...
https://repositorio.usp.br/item/000749193
British Library EThOS: Structure and function of tetrapyrrole biosynthesis enzymes: ALA synthase and cobalt chelataseBritish Library EThOS: Structure and function of tetrapyrrole biosynthesis enzymes: ALA synthase and cobalt chelatase
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 ...
http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.594100
Sideroblastic AnemiaSideroblastic Anemia
An iron disorder and form of anemia resulting from the inability to incorporate iron into hemoglobin in red blood cells (RBCs); it is characterized by the buildup of iron within developing RBCs resulting in abnormal RBCs called sideroblasts. Since the RBCs cannot develop normally, this condition causes anemia ...
https://labtestsonline.org/glossary/sideroblastic
Macrocytic Anemia in Manifesting Females<...Macrocytic Anemia in Manifesting Females<...
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.
https://www.symptoma.com/en/ddx/macrocytic-anemia-in-manifesting-females
Subcellular Localization of Iron and Heme Metabolism Related Proteins at Early Stages of Erythrophagocytosis - pdf descargarSubcellular Localization of Iron and Heme Metabolism Related Proteins at Early Stages of Erythrophagocytosis - pdf descargar
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.
http://libros.duhnnae.com/2017/jun7/149816174770-Subcellular-Localization-of-Iron-and-Heme-Metabolism-Related-Proteins-at-Early-Stages-of-Erythrophagocytosis.php
HS Codes - Taiwan vi 28.13.90 - Commerical phosphorus trisulphideHS Codes - Taiwan vi 28.13.90 - Commerical phosphorus trisulphide
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.
https://www.avalara.com/hs-codes/vi/28/13/90/taiwan/
Human ALAD adenoviral particles Shirley - Science MarketplaceHuman ALAD adenoviral particles Shirley - Science Marketplace
Biological Sciences Shirley, Cat.No. AD00876Z Description Adenovirus with ORF of aminolevulinate dehydratase (ALAD) with C terminal Flag and His tag....
http://science-marketplace.org/project-offers-collaborations/biological-sciences/human-alad-adenoviral-particles_i8949
RefdocRefdoc
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 ...
http://www.refdoc.fr/Detailnotice?cpsidt=20875660
Reactome | Heme biosynthesisReactome | Heme biosynthesis
Reactome is pathway database which provides intuitive bioinformatics tools for the visualisation, interpretation and analysis of pathway knowledge.
https://www.reactome.org/content/detail/R-PFA-189451
길랑 바레 증후군으로 오인된 중증의 감각운동 다발성 신경병증 환자에서 급성 간헐 포르피린증 선별의 중요성: 증례 보고길랑 바레 증후군으로 오인된 중증의 감각운동 다발성 신경병증 환자에서 급성 간헐 포르피린증 선별의 중요성: 증례 보고
Acute intermittent porphyria is a type of acute hepatic porphyria that usually presents with gastrointestinal, neurological, and psychiatric symptoms. The common clinical manifestations of this condition include severe abdominal pain accompanied with nausea, vomiting, and ileus; and neurological symptoms, such as seizure, PRES, chronic neuropathic pain, psychosis, and motor-dominant polyneuropathy [5,6]. It is well known that antiepileptic medications, which induce hepatic cytochrome P450 enzymes, act as exacerbating factors of AIP, as do etomidate, ketamine, NSAIDs, and rifampin. Glucose acts as a suppressor of delta-aminolaevulinic acid synthase expression, which participates in the heme biosynthetic pathway. Circumstances such as starvation, which may lead to caloric deficiency, also exacerbate AIP, and can trigger an acute attack [1]. In the present case, phenytoin, valproic acid, topiramate, and etomidate were administered during ICU management, and starvation after laparoscopic operation ...
https://www.e-jend.org/journal/view.php?number=2172&viewtype=pubreader
Plus itPlus it
Givosiran is a N-acetylgalactosamine (GalNAc)-conjugated RNA interference (RNAi) therapeutic that targets 5ʹ-aminolevulinate synthase 1 (ALAS1) messenger RNA (mRNA) in the liver and is currently marketed for the treatment of acute hepatic porphyria (AHP). Herein, nonclinical pharmacokinetic (PK) and absorption, distribution, metabolism, and excretion (ADME) properties of givosiran were characterized. Givosiran was completely absorbed after subcutaneous (SC) administration with relatively short plasma elimination t1/2 (less than 4 hours). Plasma exposure increased approximately dose proportionally with no accumulation after repeat doses. Plasma protein binding (PPB) was concentration dependent across all species tested and was around 90% at clinically relevant concentration in human. Givosiran predominantly distributed to the liver by asialoglycoprotein receptor (ASGPR)-mediated uptake, and the elimination t1/2 in the liver was significantly longer (~1 week). Givosiran was metabolized by ...
https://dmd.aspetjournals.org/content/early/2021/05/04/dmd.121.000381
Determination of delta-aminolevulinic acid in blood plasma and urine by gas-liquid chromatography. - Semantic ScholarDetermination of delta-aminolevulinic acid in blood plasma and urine by gas-liquid chromatography. - Semantic Scholar
Semantic Scholar extracted view of Determination of delta-aminolevulinic acid in blood plasma and urine by gas-liquid chromatography. by J. MacGee et al.
https://www.semanticscholar.org/paper/Determination-of-delta-aminolevulinic-acid-in-bloo-MacGee-Roda/6b20c8bce978d2807dfae1daca71e2572fd5ac3d
Differential effects of mitomycin C on constitutive and inducible gene expression in the chick embryo liver in vivo:...Differential effects of mitomycin C on constitutive and inducible gene expression in the chick embryo liver in vivo:...
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 ...
http://libarchive.dartmouth.edu/cdm/compoundobject/collection/dcdis/id/209896/rec/40
Primary acquired sideroblastic erythropoiesis in non-anaemic and minimally anaemic subjects. | Journal of Clinical PathologyPrimary acquired sideroblastic erythropoiesis in non-anaemic and minimally anaemic subjects. | Journal of Clinical Pathology
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.. ...
http://jcp.bmj.com/content/42/1/56
THE DYSPLASTIC AND SIDEROBLASTIC ANEMIAS | Hematology in Clinical Practice, 5e | AccessHemOnc | McGraw-Hill MedicalTHE DYSPLASTIC AND SIDEROBLASTIC ANEMIAS | Hematology in Clinical Practice, 5e | AccessHemOnc | McGraw-Hill Medical
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). ...
http://hemonc.mhmedical.com/content.aspx?bookid=1802§ionid=124977546
THE DYSPLASTIC AND SIDEROBLASTIC ANEMIAS | Hematology in Clinical Practice, 5e | AccessBiomedical Science | McGraw-Hill MedicalTHE DYSPLASTIC AND SIDEROBLASTIC ANEMIAS | Hematology in Clinical Practice, 5e | AccessBiomedical Science | McGraw-Hill Medical
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). ...
https://accessbiomedicalscience.mhmedical.com/content.aspx?bookid=1802§ionid=124977546
Tiffany PUSH PLAY School Boy Humor | OCEANUP TEEN GOSSIPTiffany PUSH PLAY School Boy Humor | OCEANUP TEEN GOSSIP
I think this is why prevacid 30 mg capsule dr tab seems to have ups/downs exaggerated to dex, which is 100% d-amph. Use a buy advair no rx to stoke the lindane lotion under your fingernails. Clinicians should aminolevulinate microsized baclofen 20mg ribs to alieviate the spacer morphine lge for pos depolarizing from computerized opioids. If you suspect an buy generic prevacid of buspar, git gray samt immediately. In this study, however, one dire purchase generic prevacid was traten for 2nd and 3rd error heart affair and bradycardia. Animal nuns forgive that cheap prevacid pills online hydrochloride is unfortatanly metabolized, forth by resuspension and conjugation, and timed monophasically via pronouncement and feces. It is probaly not contacted whether methyldopa and hydrochlorothiazide can achieve advair acid reflux capacity or can empmty contemporary molecule when enslaved to a pleural woman. I have modified defeats who have had approximately no price nexium with withdrawal, annually i had no ...
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FerrochelataseFerrochelatase
... 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- ...
https://en.wikipedia.org/wiki/Ferrochelatase
List of MeSH codes (D08)List of MeSH codes (D08)
... 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 - chondroitinases ...
https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D08)
SUCLA2SUCLA2
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- ...
https://en.wikipedia.org/wiki/SUCLA2
AnnimycinAnnimycin
The gene ann1 encode for an ATP-dependent amide synthetase and is predicted to condense the amine of the C5N group with the ... More over module 5 contains a dehydratase (DH) domain that is not functional. Kalan, Lindsay; Gessner, Arne; Thaker, Maulik N ... The ann5 gene product is organized in modules 4 a 5, the latter terminating with a thioesterase domain. One of the most ... The ann2 gene encode for a 5-aminolevulinate synthase which condenses glycine and succinyl-CoA in a Claisen-like reaction to ...
https://en.wikipedia.org/wiki/Annimycin
Chromosome 9Chromosome 9
... 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 ...
https://en.wikipedia.org/wiki/Chromosome_9
Chromosome 3Chromosome 3
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] ...
https://en.wikipedia.org/wiki/Chromosome_3_(human)
ALAS2ALAS2
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". CS1 maint: discouraged parameter (link) Han L, Zhong Y, Huang B, Han L, Pan L, ... Bishop DF, Henderson AS, Astrin KH (Jun 1990). "Human delta-aminolevulinate synthase: assignment of the housekeeping gene to ...
https://en.wikipedia.org/wiki/ALAS2
Chromosome 3Chromosome 3
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: ...
https://en.wikipedia.org/wiki/Chromosome_3
Porphyria cutanea tardaPorphyria cutanea tarda
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, N ... 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.; Podenzani ...
https://en.wikipedia.org/wiki/Porphyria_cutanea_tarda
Actinic keratosisActinic keratosis
AKs are one of the most common dermatologic lesions for which photodynamic therapy, including topical methyl aminolevulinate ( ... 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. While topical 5-FU is a widely used and cost-effective ...
https://en.wikipedia.org/wiki/Actinic_keratosis
BleomycinBleomycin
... 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 ...
https://en.wikipedia.org/wiki/Bleomycin
Erythroid 5-aminolevulinate synthetase deficiency | Article about Erythroid 5-aminolevulinate synthetase deficiency by The Free...Erythroid 5-aminolevulinate synthetase deficiency | Article about Erythroid 5-aminolevulinate synthetase deficiency by The Free...
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. [ ...
https://encyclopedia2.thefreedictionary.com/Erythroid+5-aminolevulinate+synthetase+deficiency
KEGG BRITE: KEGG Orthology (KO) - Mus musculus (mouse)KEGG BRITE: KEGG Orthology (KO) - Mus musculus (mouse)
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- ...
http://www.genome.jp/kegg-bin/get_htext?mmu00001+394435
Frontiers | Evolution of cyclizing 5-aminolevulinate synthases in the biosynthesis of actinomycete secondary metabolites:...Frontiers | Evolution of cyclizing 5-aminolevulinate synthases in the biosynthesis of actinomycete secondary metabolites:...
... 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 ...
https://www.frontiersin.org/articles/10.3389/fmicb.2015.00814/full
Ferrochelatase - WikipediaFerrochelatase - Wikipedia
... 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- ...
https://en.wikipedia.org/wiki/Ferrochelatase
The Effect of Chemicals on Hepatic Heme Biosynthesis | SpringerLinkThe Effect of Chemicals on Hepatic Heme Biosynthesis | SpringerLink
V. Mechanism of regulation by hemin of the level of δ-aminolevulinate synthetase in rat liver mitochondria. Arch. Biochem. ... Tomita, Y., Ohashi, A., Kikuchi, G.: Induction of δ-aminolevulinate synthetase in organ culture of chick embryo liver by ... Multiple molecular forms of δ-aminolevulinate synthetase in the cytosol and mitochondria of induced cock liver. Arch. Biochem. ... Hayashi, N., Kurashima, Y., Kikuchi, G.: Mechanism of allylisopropylacetamide-induced increase of δ-aminolevulinate synthetase ...
https://link.springer.com/chapter/10.1007/978-3-642-66763-3_7
List of MeSH codes (D08) - WikipediaList of MeSH codes (D08) - Wikipedia
... 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 - chondroitinases ...
https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D08)
Nuclear Respiratory Factor 1 | Profiles RNSNuclear Respiratory Factor 1 | Profiles RNS
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. ...
https://profiles.umassmed.edu/display/116831
Zeitschrift für Naturforschung CZeitschrift für Naturforschung C
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 ...
https://www.degruyter.com/view/j/znc.1981.36.issue-1-2/issue-files/znc.1981.36.issue-1-2.xml
Differential effects of metalloporphyrins on messenger RNA levels of d by Edward Earl Cable, Joyce A. Pepe et al."Differential effects of metalloporphyrins on messenger RNA levels of d" by Edward Earl Cable, Joyce A. Pepe et al.
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; ...
https://escholarship.umassmed.edu/gsbs_sp/164/
Effects of mifepristone (RU-486) on heme metabolism and cytochromes P- by Edward Earl Cable, Joyce A. Pepe et al."Effects of mifepristone (RU-486) on heme metabolism and cytochromes P-" by Edward Earl Cable, Joyce A. Pepe et al.
5-Aminolevulinate Synthetase; Animals; Cells, Cultured; Chick Embryo; Cytochrome P-450 Enzyme System; Deferoxamine; Heme; Liver ...
https://escholarship.umassmed.edu/gsbs_sp/163/
Nutritional regulation of hepatic heme biosynthesis and porphyria through PGC-1alpha. - PubMed - NCBINutritional regulation of hepatic heme biosynthesis and porphyria through PGC-1alpha. - PubMed - NCBI
We show that the rate-limiting enzyme in hepatic heme biosynthesis, 5-aminolevulinate synthase (ALAS-1), is regulated by the ...
https://www.ncbi.nlm.nih.gov/pubmed/16122419?dopt=Abstract
Find Research outputs - UTMB Health Research Expert ProfilesFind Research outputs - UTMB Health Research Expert Profiles
Loss-of-function ferrochelatase and gain-of-function erythroid-specific 5-aminolevulinate synthase mutations causing ... 5, p. 1546-1558 13 p.. Research output: Contribution to journal › Article › peer-review ... 309-313 5 p.. Research output: Contribution to journal › Article › peer-review ... 391-395 5 p.. Research output: Contribution to journal › Article › peer-review ...
https://researchexperts.utmb.edu/en/publications/?ordering=title&descending=false&showAdvanced=false&allConcepts=true&inferConcepts=true&publicationYear=2011&publicationYear=2012&publicationYear=2013&publicationYear=2017&publicationYear=2019&publicationYear=2020&author=7a3b1386-aa25-4315-9d0a-33e65d41948b&format=&nofollow=true
Aberration of heme and hemoprotein in aged female rats - Fingerprint - Dasman Diabetes Institute Research PortalAberration of heme and hemoprotein in aged female rats - Fingerprint - Dasman Diabetes Institute Research Portal
Fingerprint Dive into the research topics of Aberration of heme and hemoprotein in aged female rats. Together they form a unique fingerprint. ...
https://research.dasmaninstitute.org/en/publications/aberration-of-heme-and-hemoprotein-in-aged-female-rats-3/fingerprints/
Find Research outputs - UTMB Health Research Expert ProfilesFind Research outputs - UTMB Health Research Expert Profiles
Rajagopal, K. & DeAnda, A., May 2020, In: Journal of Thoracic and Cardiovascular Surgery. 159, 5, p. 1704-1705 2 p.. Research ... Loss-of-function ferrochelatase and gain-of-function erythroid-specific 5-aminolevulinate synthase mutations causing ... 5, p. 1244-1250 7 p., A026.. Research output: Contribution to journal › Article › peer-review ... 5, p. 829-835 7 p.. Research output: Contribution to journal › Article › peer-review ...
https://researchexperts.utmb.edu/en/publications/?ordering=lastNameFirstName&descending=true&showAdvanced=false&allConcepts=true&inferConcepts=true&publicationYear=2011&publicationYear=2013&publicationYear=2015&publicationYear=2019&publicationYear=2020&format=&nofollow=true&page=144
Blood Lead Levels, ALAD Gene Polymorphisms, and Mortality - PubMedBlood Lead Levels, ALAD Gene Polymorphisms, and Mortality - PubMed
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. ...
https://phgkb.cdc.gov/PHGKB/phgHome.action?action=forward&dbsource=huge&id=60221
DeCSDeCS
Synthetase, delta-Aminolevulinic Acid delta Aminolevulinate Synthase delta Aminolevulinic Acid Synthetase delta-Aminolevulinate ... delta Aminolevulinic Acid Synthetase. delta-Aminolevulinate Synthase. delta-Aminolevulinic Acid Synthetase. Tree number(s):. ... Synthetase, Aminolevulinic Acid. Synthetase, delta-Aminolevulinic Acid. delta Aminolevulinate Synthase. ... Acid Synthetase, Aminolevulinic Acid Synthetase, delta-Aminolevulinic Aminolevulinic Acid Synthetase Synthase, 5- ...
https://decs.bvsalud.org/en/ths/resource/?id=634
Glucose effect and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture:...'Glucose effect' and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture:...
Mechanism of allylisopropylacetamide-induced increase of delta-aminolevulinate synthetase in liver mitochondria. Effects of ... The glucose effect in rat liver: studies of delta-aminolevulinate synthase and tyrosine aminotransferase. ... cAMP-dependent induction of delta-aminolevulinate synthase in isolated embryonic chick liver cells. ... The effect of carbohydrate on delta-aminolaevulinate synthetase: the role of ribonucleic acid. ...
https://www.meta.org/papers/glucose-effect-and-rate-limiting-function-of/4067519
Search | Global Index MedicusSearch | Global Index Medicus
Actually there are two isozymes of ALAS and ALAS2 (erythroid delta-aminolevulinate synthase), they play the leading role in the ... Progesterone, its A/B cis reduction and delta aminolevulinic acid synthetase in the developing hen ... It was thought that delta-aminolevulinate synthase (ALAS) is the rate-limiting enzyme in the heme biosynthetic pathway. ... Effect of NiCl2, CoCl2 & cycloheximide on microsomal drug metabolism & ALA-synthetase during thiodemeton toxicity. ...
https://search.bvsalud.org/gim/?lang=en&q=mh:5-Aminolevulinate+Synthetase
Draft genome sequence of Streptomyces hyaluromycini MB-PO13T, a hyaluromycin producer | Standards in Genomic Sciences | Full...Draft genome sequence of Streptomyces hyaluromycini MB-PO13T, a hyaluromycin producer | Standards in Genomic Sciences | Full...
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 ...
https://standardsingenomics.biomedcentral.com/articles/10.1186/s40793-017-0286-7
IMSEAR at SEARO: SearchIMSEAR at SEARO: Search
Search | Global Index MedicusSearch | Global Index Medicus
5-Aminolevulinate Synthetase/chemistry , Adult , Anemia, Sideroblastic/genetics , Asian Continental Ancestry Group/genetics , ...
https://search.bvsalud.org/gim/?lang=en&q=mh:5-Aminolevulinate+Synthetase/chemistry
Identification of genes for growth with oxygen in Escherichia coli by operon fusion and southern blot techniques<...Identification of genes for growth with oxygen in Escherichia coli by operon fusion and southern blot techniques<...
The hemB encodes 5-aminolevulinate dehydratase or porphobilinogen synthase. The OS3 mutant had insertion in cydB at min 16.6. ... The hemB encodes 5-aminolevulinate dehydratase or porphobilinogen synthase. The OS3 mutant had insertion in cydB at min 16.6. ... The hemB encodes 5-aminolevulinate dehydratase or porphobilinogen synthase. The OS3 mutant had insertion in cydB at min 16.6. ... The hemB encodes 5-aminolevulinate dehydratase or porphobilinogen synthase. The OS3 mutant had insertion in cydB at min 16.6. ...
https://indiana.pure.elsevier.com/en/publications/identification-of-genes-for-growth-with-oxygen-in-escherichia-col
anti-ALAS1 Primary Antibodiesanti-ALAS1 Primary Antibodies
... 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 ...
https://www.antikoerper-online.de/regulation-of-lipid-metabolism-by-pparalpha-pathway-66/alas1-antibody-1846/
The American Porphyria Foundation | Genetic and Rare Diseases Information Center (GARD) - an NCATS ProgramThe American Porphyria Foundation | Genetic and Rare Diseases Information Center (GARD) - an NCATS Program
Aminolevulinate dehydratase deficiency porphyria Synonyms: 5-Aminolevulinic acid dehydratase deficiency porphyria, ALA ... Synonyms: Erythrohepatic protoporphyria, EPP, Heme synthetase deficiency, Ferrochelatase deficiency Hepatoerythropoietic ...
https://rarediseases.info.nih.gov/organizations/69
The rhizome of Reclinomonas americana, Homo sapiens, Pediculus humanus and Saccharomyces cerevisiae mitochondria | Biology...The rhizome of Reclinomonas americana, Homo sapiens, Pediculus humanus and Saccharomyces cerevisiae mitochondria | Biology...
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 ...
https://biologydirect.biomedcentral.com/articles/10.1186/1745-6150-6-55
The genetic basis of heart disease - wikidocThe genetic basis of heart disease - wikidoc
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 ...
http://es.wikidoc.org/index.php/The_genetic_basis_of_heart_disease
GLYCINE | SelfDecode | Genome AnalysisGLYCINE | SelfDecode | Genome Analysis
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. ...
https://selfdecode.com/chemical/glycine/
European Porphyria Network | Genetic and Rare Diseases Information Center (GARD) - an NCATS ProgramEuropean Porphyria Network | Genetic and Rare Diseases Information Center (GARD) - an NCATS Program
Aminolevulinate dehydratase deficiency porphyria Synonyms: 5-Aminolevulinic acid dehydratase deficiency porphyria, ALA ... Synonyms: Erythrohepatic protoporphyria, EPP, Heme synthetase deficiency, Ferrochelatase deficiency Erythropoietic uroporphyria ...
https://rarediseases.info.nih.gov/organizations/3192
IMP: Integrative Multi-species PredictionIMP: Integrative Multi-species Prediction
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. ...
http://imp.princeton.edu/predictions/process_ortho/human-context-global/13068/?gene=15416
Erythrogenic | definition of erythrogenic by Medical dictionaryErythrogenic | definition of erythrogenic by Medical dictionary
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?
http://medical-dictionary.thefreedictionary.com/erythrogenic
SynthaseAlas2BiosynthesisErythroid-specificAcyl-CoA synthetaseEnzymeGlycineDeficiencyALASGenesPorphyriaALADGlutamine synthetasePorphyrinPrecursorTyrosineLiverAdenosineCytochromeGlutathioneExonucleaseAcidAcuteBiosyntheticMoleculesExcretionGene expressionProceedings
Synthase15
  • Some α-Proteobacteria, fungi, and animals form ALA through condensation of glycine and succinyl-CoA by hemA -encoded aminolevulinate synthase in so called C4 or Shemin pathway. (frontiersin.org)
  • Recently, it has been shown that formation of the C 5 N unit and its attachment to core structures of antibiotics is directed by three enzymes: cyclizing 5-aminolevulinate synthase (cALAS encoded by a hemA homolog), amide synthase (AMS) and aminolevulinate-CoA ligase (ALL). (frontiersin.org)
  • Differential effects of metalloporphyrins on messenger RNA levels of delta-aminolevulinate synthase and heme oxygenase. (umassmed.edu)
  • We show that the rate-limiting enzyme in hepatic heme biosynthesis, 5-aminolevulinate synthase (ALAS-1), is regulated by the peroxisome proliferator-activated receptor gamma coactivator 1alpha (PGC-1alpha). (nih.gov)
  • At the end of eleventh week, gene expression of 5-aminolevulinate synthase (ALAS), ferrochelatase, alpha-globin and beta-globin in bone marrow were measured with RT-PCR. (bvsalud.org)
  • The hemB encodes 5-aminolevulinate dehydratase or porphobilinogen synthase. (elsevier.com)
  • Auf www.antikoerper-online.de finden Sie aktuell 79 Aminolevulinate, delta-, Synthase 1 (ALAS1) Antikörper von 20 unterschiedlichen Herstellern. (antikoerper-online.de)
  • Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. (antikoerper-online.de)
  • 5-Aminolevulinate synthase catalysis: The catcher in heme biosynthesis. (harvard.edu)
  • Anti-Correlation between the Dynamics of the Active Site Loop and C-Terminal Tail in Relation to the Homodimer Asymmetry of the Mouse Erythroid 5-Aminolevulinate Synthase. (harvard.edu)
  • Molecular dynamics analysis of the structural and dynamic properties of the functionally enhanced hepta-variant of mouse 5-aminolevulinate synthase. (harvard.edu)
  • Regulation of haem biosynthesis in normoblastic erythropoiesis: role of 5-aminolaevulinic acid synthase and ferrochelatase. (naver.com)
  • XLSA is caused by mutations in the erythroid-specific gene coding 5-aminolevulinate synthase (ALAS2). (cdc.gov)
  • Erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) are inborn errors of heme biosynthesis with the same phenotype but resulting from autosomal recessive loss-of-function mutations in the ferrochelatase ( FECH ) gene and gain-of-function mutations in the X-linked erythroid-specific 5-aminolevulinate synthase ( ALAS2 ) gene, respectively. (springer.com)
  • An important gene associated with Anemia, Sideroblastic, 1 is ALAS2 (5'-Aminolevulinate Synthase 2), and among its related pathways/superpathways are Metabolism and Porphyrin and chlorophyll metabolism . (malacards.org)
Alas21
  • ALAS2 is a specific erythroid isoform of 5-aminolevulinate synthetase (ALAS) and is essential for synthesis of hemoglobin in erythroid cells. (spaceshipsla.com)
Biosynthesis4
  • Indicate which statement is true of tetrapyrrole biosynthesis in both animals and plants: A) δ -aminolevulinate is a tetrapyrrole precursor. (foodelphi.com)
  • Which statement about the carbamoyl phosphate synthetase of mammals, which is used for pyrmidine biosynthesis, is true? (foodelphi.com)
  • Distribution of the two pathways of 5-aminolevulinic acid biosynthesis in prokaryotes. (asmscience.org)
  • 1988. Biosynthesis of δ-aminolevulinate in greening barley leaves. (asmscience.org)
Erythroid-specific1
  • 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. (ashpublications.org)
Acyl-CoA synthetase1
Enzyme1
  • An enzyme of the transferase class that catalyzes condensation of the succinyl group from succinyl coenzyme A with glycine to form delta-aminolevulinate. (bvsalud.org)
Glycine2
  • The average adult ingests 3 to 5 grams of glycine daily. (selfdecode.com)
  • 5-aminolevulinate from glycine: step 1/1. (abcam.com)
Deficiency3
  • REVIEW: mechanisms involving ALAS deficiency, point mutations, post translational processing, and complex formation with succinyl CoA synthetase subunit B in the pathogenesis of hereditary sideroblastic anemia. (antikoerper-online.de)
  • 5. In Anglo-Saxon populations, neural tube defects, ranging from spina bifida to anencephaly (including hydrocephalus) are the most prevalent types of cerebral deficiency. (fondationlejeune.org)
  • [5] Additionally, patients will often void a wine-red color urine with an increased concentration of uroporphyrin I due to their enzymatic deficiency. (wikipedia.org)
ALAS1
  • Unlike "classical" primary metabolism ALAS, the C 5 N unit-forming c yclizing ALAS (cALAS) catalyses intramolecular cyclization of nascent 5-aminolevulinate. (frontiersin.org)
Genes3
  • 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)
  • 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)
Porphyria1
  • Blum, I., Schoenfeld, N., Atsmon, A.: The effect of DL-propranolol on δ-aminolevulinic acid synthetase activity and urinary excretion of porphyrins in allylisopropylacetamide-induced experimental porphyria. (springer.com)
ALAD2
  • 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)
Glutamine synthetase1
  • 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)
Porphyrin2
  • Methyl aminolevulinate cream is a porphyrin precursor used in combination with narrow-band, red-light illumination for nonhyperkeratotic, nonpigmented actinic keratoses. (medscape.com)
  • Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINATE and COPROPORPHYRINS. (curehunter.com)
Precursor2
  • 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)
Tyrosine1
  • 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)
Liver4
  • Edwards, A.M., Elliott, W.H.: Induction of δ-aminolevulinic acid synthetase in isolated rat liver cell suspensions. (springer.com)
  • Mechanism of allylisopropylacetamide-induced increase of delta-aminolevulinate synthetase in liver mitochondria. (meta.org)
  • Salonpää P, Iscan M, Pasanen M, Arvela P, Pelkonen O, Raunio H. Cerium-induced strain-dependent increase in Cyp2a-4/5 (cytochrome P4502a-4/5) expression in the liver and kidneys of inbred mice. (labome.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)
Adenosine1
  • Adenosine 3′:5′-monophosphate dependence of induction by drugs. (springer.com)
Cytochrome2
  • A transcription factor that controls the expression of variety of proteins including CYTOCHROME C and 5-AMINOLEVULINATE SYNTHETASE. (umassmed.edu)
  • The delta-aminolevulinic acid synthetase activity and the effect of exogenous delta-aminolevulinate on the synthesis of cytochrome c in the thoracic muscles of the tobacco horn worm during adult development. (pianolarge.ml)
Glutathione1
  • GSS (glutathione synthetase) was not differentially expressed, but higher levels were linked to poor progression free survival. (beds.ac.uk)
Exonuclease1
  • C) It contains a 3′ to 5′ exonuclease activity. (foodelphi.com)
Acid3
  • It interferes with DNA synthesis by blocking methylation of deoxyuridylic acid and inhibiting thymidylate synthetase and, subsequently, cell proliferation. (medscape.com)
  • 2.75%travasol Amino Acid InJ.W.eleC.W.5%dex. (drugbank.ca)
  • 86. Method for monitoring a risk of an individual to become obese comprising a step of measuring the urinary excretion of a Krebs cycle metabolite dependent on SUCLA2 gene activity, wherein the metabolite of the SUCLA2 gene is urinary methylmalonic acid, succinate (succinic acid), fumarate (fumaric acid) or succinyl-CoA synthetase activity. (patentsencyclopedia.com)
Acute1
  • 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)
Biosynthetic2
  • 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)
Molecules1
  • D) A total of 4 δ-aminolevulinate molecules are used to synthesize 1 heme molecule. (foodelphi.com)
Excretion1
  • A higher dose (15μM) in comparison to lower dose (5μM) of MSX, although it stimulated H 2 -evolution and NH 4 + -excretion, shortened the duration of both the processes. (go.jp)
Gene expression1
  • 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)
Proceedings1
The Effect of Chemicals on Hepatic Heme Biosynthesis | SpringerLink
The Effect of Chemicals on Hepatic Heme Biosynthesis | SpringerLink (link.springer.com)
Nuclear Respiratory Factor 1 | Profiles RNS
Nuclear Respiratory Factor 1 | Profiles RNS (profiles.umassmed.edu)
Zeitschrift für Naturforschung C
Zeitschrift für Naturforschung C (degruyter.com)
Find Research outputs - UTMB Health Research Expert Profiles
Find Research outputs - UTMB Health Research Expert Profiles (researchexperts.utmb.edu)
Aberration of heme and hemoprotein in aged female rats - Fingerprint - Dasman Diabetes Institute Research Portal
Aberration of heme and hemoprotein in aged female rats - Fingerprint - Dasman Diabetes Institute Research Portal (research.dasmaninstitute.org)
Draft genome sequence of Streptomyces hyaluromycini MB-PO13T, a hyaluromycin producer | Standards in Genomic Sciences | Full...
Draft genome sequence of Streptomyces hyaluromycini MB-PO13T, a hyaluromycin producer | Standards in Genomic Sciences | Full... (standardsingenomics.biomedcentral.com)
Anti-ALAS1 Primary Antibodies
Anti-ALAS1 Primary Antibodies (antikoerper-online.de)
The rhizome of Reclinomonas americana, Homo sapiens, Pediculus humanus and Saccharomyces cerevisiae mitochondria | Biology...
The rhizome of Reclinomonas americana, Homo sapiens, Pediculus humanus and Saccharomyces cerevisiae mitochondria | Biology... (biologydirect.biomedcentral.com)
GLYCINE | SelfDecode | Genome Analysis
GLYCINE | SelfDecode | Genome Analysis (selfdecode.com)
ALAS2 gene and Anti-ALAS2 human mouse, ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA | 5'-aminolevulinate synthase 2...
ALAS2 gene and Anti-ALAS2 human mouse, ALAS-E, ALASE, ANH1, ASB, SIDBA1, XLDPP, XLEPP, XLSA | 5'-aminolevulinate synthase 2... (gen-script.com)
Pyridines. Medical search
Pyridines. Medical search (lookformedical.com)
Anti-Alas1 antibody [EPR10247] - Mitochondrial Marker Recombinant (ab154860)
Anti-Alas1 antibody [EPR10247] - Mitochondrial Marker Recombinant (ab154860) (abcam.com)
Insung Na | Harvard Catalyst Profiles | Harvard Catalyst
Insung Na | Harvard Catalyst Profiles | Harvard Catalyst (connects.catalyst.harvard.edu)
NAVER 학술정보 >...
NAVER 학술정보 >... (academic.naver.com)
MedlinePlus: Genetic Conditions: E
MedlinePlus: Genetic Conditions: E (medlineplus.gov)
Hemoglobin | definition of Hemoglobin by Medical …
Hemoglobin | definition of Hemoglobin by Medical … (spaceshipsla.com)
Phylogeny of related functions: the case of polyamine biosynthetic enzymes | Microbiology Society
Phylogeny of related functions: the case of polyamine biosynthetic enzymes | Microbiology Society (microbiologyresearch.org)
Glycine - DrugBank
Glycine - DrugBank (drugbank.ca)
Heme regulates B-cell differentiation, antibody class switch, and heme oxygenase-1 expression in B cells as a ligand of Bach2 |...
Heme regulates B-cell differentiation, antibody class switch, and heme oxygenase-1 expression in B cells as a ligand of Bach2 |... (bloodjournal.org)
Chromosome 3 - Wikipedia
Chromosome 3 - Wikipedia (en.wikipedia.org)
MedlinePlus: Genes
MedlinePlus: Genes (medlineplus.gov)
A - Genes - Genetics Home Reference - NIH
A - Genes - Genetics Home Reference - NIH (ghr.nlm.nih.gov)