• Asparagine synthetase deficiency is a condition that causes neurological problems in affected individuals starting soon after birth. (medlineplus.gov)
  • Most individuals with asparagine synthetase deficiency have exaggerated reflexes (hyperreflexia) and weak muscle tone (hypotonia). (medlineplus.gov)
  • People with asparagine synthetase deficiency may have an exaggerated startle reaction (hyperekplexia) to unexpected stimuli. (medlineplus.gov)
  • People with asparagine synthetase deficiency typically do not survive past childhood. (medlineplus.gov)
  • Asparagine synthetase deficiency is thought to be a rare condition. (medlineplus.gov)
  • Asparagine synthetase deficiency is caused by mutations in a gene called ASNS . (medlineplus.gov)
  • This gene provides instructions for making an enzyme called asparagine synthetase. (medlineplus.gov)
  • Mutations in the ASNS gene that cause asparagine synthetase deficiency lead to a decrease or loss of functional enzyme. (medlineplus.gov)
  • As a result, brain cells in people with asparagine synthetase deficiency have a shortage (deficiency) of this amino acid. (medlineplus.gov)
  • The exact effect of asparagine synthetase deficiency on brain cells is unknown, but because of the severe features of this condition, it is clear that asparagine is necessary for normal brain development. (medlineplus.gov)
  • Asparagine synthetase (or aspartate-ammonia ligase) is a chiefly cytoplasmic enzyme that generates asparagine from aspartate. (wikipedia.org)
  • This amidation reaction is similar to that promoted by glutamine synthetase. (wikipedia.org)
  • Above average presence of asparagine synthetase in certain leukemia strains has been linked to be a significant contributing factor of chemotherapy resistance, particularly to the chemotherapy drug, L-asparaginase. (wikipedia.org)
  • Escherichia coli derived asparagine synthetase is a dimeric protein with each subunit folding into two distinct domains. (wikipedia.org)
  • Structural characterization of asparagine synthetase from mammalian sources have been difficult due to the low abundance and instability of the enzyme during purification procedures. (wikipedia.org)
  • Using information from Escherichia coli derived asparagine synthetase, some basic mechanisms of the enzyme have been understood. (wikipedia.org)
  • Assimilation of this nitrogen into asparagine for use in nitrogen recycling, transport, and storage is an essential process for plant development, making asparagine synthetase vital to maintaining these asparagine reserves. (wikipedia.org)
  • Specific events in development which depend on asparagine synthetase are nitrogen mobilization in germinating seeds, nitrogen recycling and flow in vegetative cells in response to biotic and abiotic stresses, and nitrogen remobilization from source to sink organs. (wikipedia.org)
  • In mammals, asparagine synthetase expression has been found to be linked to cell growth, and its mRNA content is linked to changes in the cell cycle. (wikipedia.org)
  • Hamster BHK ts11 cells produce an inactive asparagine synthetase enzyme, and this loss of asparagine synthetase activity directly led to cell cycle arrest in the cells as a consequence of a depletion of cellular asparagine. (wikipedia.org)
  • Upregulation of asparagine synthetase mRNA was observed as well in these hamster cells. (wikipedia.org)
  • Other experiments demonstrated that quiescent rat thyroid cells entering S phase as a result of thyroid-stimulating hormone treatment was matched with a concurrent increase in asparagine synthetase mRNA content. (wikipedia.org)
  • There seem to be two major groups of asparagine synthetase: Majority of prokaryotic isolated enzymes (asnA) utilize ammonia as the sole nitrogen source. (wikipedia.org)
  • The lack of ammonia-dependent asparagine synthetase in eukaryotes is presumably because of the need to maintain cellular concentrations of ammonia at very low levels. (wikipedia.org)
  • The particularly low-level expression of asparagine synthetase in primary acute lymphoblastic leukemia (ALL) and numerous ALL cell lines, as compared to that of normal cells, makes asparagine depletion an effective method of treatment due to the cells' unusual dependency on circulating serum asparagine as a necessary nutrition for growth. (wikipedia.org)
  • As a result, L-asparaginase is a common chemotherapy drug utilized in the treatment of ALL and may have applications in other asparagine synthetase negative cancers, such as lymphomas, due to its aspariginase activity to deplete serum asparagine. (wikipedia.org)
  • Asparagine synthetase (ASNS) converts aspartate and glutamine to asparagine and glutamate in an ATP-dependent reaction. (nih.gov)
  • Thus far, 15 unique mutations in the ASNS gene have been clinically associated with asparagine synthetase deficiency (ASD). (nih.gov)
  • The early hope that L-asparaginase would be a breakthrough in medical treatment, with selective toxic effects based on the qualitative presence or absence of a specific enzyme (asparagine synthetase), has not been realized. (nih.gov)
  • Mechanism = Tumor cells lack asparagine synthetase and thus require exogenous sources of L-Asparagine. (thehematologist.org)
  • however, when present on primary amines or thiols, principally lysine and cysteine, they can undergo a second reaction with certain accessible amino acids and form cross-links. (medscape.com)
  • They consist of amino acids and perform multitude of functions - from acting as scaffolding and structural elements, to transporting molecules, driving DNA replication and catalysing metabolic reactions. (ed.ac.uk)
  • During processing, salts, other amino acids than asparagine and asparaginase may be added to lower acrylamide formation in various baked products. (slu.se)
  • Acrylamide appears to form as a result of a reaction between specific amino acids, including asparagine, and sugars found in foods reaching high temperatures during cooking processes. (bakeryandsnacks.com)
  • We extract representative amino acids with oxygen-atom-containing sidechains (i.e., tyrosine, serine, or threonine) hydrogen-bonded to the nitrogen/oxygen-atom-containing sidechains of asparagine or glutamine from high-resolution (1.5 Å or better) X-ray crystal structures in the Protein Data Bank. (aiche.org)
  • Of note is the presence of an asparagine in the active centre, in a position likely to interact with the acetyl NH group that, in all other known families of glycosidase using this mechanism, is an aspartate or glutamate residue. (rcsb.org)
  • "The enzyme converts one of the precursors of acrylamide, asparagines, into another naturally occurring amino acid, aspartate," ​ DSM said. (bakeryandsnacks.com)
  • In the Maillard reaction, asparagine gets converted into acrylamide. (novozymes.com)
  • That means it can't be converted into acrylamide in the Maillard reaction. (novozymes.com)
  • Cooking these foods at high temperatures with little moisture results in the Maillard reaction. (novozymes.com)
  • Acrylamide forms as part of a chemical reaction, known as the Maillard reaction, which contributes to the aroma, taste, and color of cooked foods. (nih.gov)
  • Acrylamide is one of the hundreds of chemicals that can form during the Maillard reaction. (nih.gov)
  • At temperatures above 100°C, the amino acid asparagine--abundant in potatoes and cereal grains--bonds with 'reducing' sugars like glucose according to the Maillard reaction. (the-scientist.com)
  • In the acrylamide pathway, a Maillard product called N-glycoside cleaves at a carbon-nitrogen bond, yielding the carbon skeleton and terminal amide group of asparagine, which transitions. (the-scientist.com)
  • Known as the Maillard reaction, this process is responsible for the brown color and tasty flavor of baked, fried and toasted foods. (foodnavigator-usa.com)
  • Free asparagine is not incorporated into proteins and might thus easily react in the Maillard reaction and form acrylamide. (slu.se)
  • The process is known as the Maillard reaction. (bakeryandsnacks.com)
  • Acrylamide, a water-soluble α,β-unsaturated amide, is a contaminant in baked and fried starchy foods, including french fries, potato chips, and bread, as a result of Maillard reactions involving asparagine and reducing sugars. (nih.gov)
  • Tests show that when the amino acid is heated, it reactswith sugar to create acrylamide, a process called the Maillard reaction. (foodnavigator-usa.com)
  • The tunnel that links the two active sites allows for the passage of an ammonia molecule to act as a common intermediate to couple the two half-reactions carried out in the independent active sites of the enzyme. (wikipedia.org)
  • Asparaginase is an enzyme that hydrolyses an amino acid, asparagine, to aspartic acid by hydrolyzing the amide in free asparagine. (canada.ca)
  • The purpose of this enzyme is to reduce the presence of asparagine. (prospecbio.com)
  • So, this enzyme has dual functions, but both revolve around taking asparagine and converting into less harmful things. (prospecbio.com)
  • Researchers have been studying this family of enzymes for many years now, but a lack of information related to natural substrates and the diversity of roles the enzymes play has made it difficult to pinpoint the specific mechanisms involved in reactions that are catalysed by this enzyme group. (prospecbio.com)
  • RYLAZE is an asparagine specific enzyme indicated as a component of a multi-agent chemotherapeutic regimen for the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in adult and pediatric patients 1 month or older who have developed hypersensitivity to E. coli -derived asparaginase. (nih.gov)
  • Casparaginase pegol-mknl is an enzyme that speeds up the breakdown of asparagine in the blood into aspartic acid and ammonia. (oncolink.org)
  • succinate reaction occurs via a histidine with a phosphoramidate arm via the enzyme succinyl thiokinase. (editthis.info)
  • ASPARLAS is an asparagine specific enzyme indicated as a component of a multi-agent chemotherapeutic regimen for the treatment of acute lymphoblastic leukemia in pediatric and young adult patients age 1 month to 21 years. (nih.gov)
  • [ 17 ] Additionally, the protein N-terminal amine can be converted to a stable 4-imidazolidione adduct [ 17 ] and a Mannich reaction can covalently join proximal tyrosine and arginine residues. (medscape.com)
  • Asparaginase has no activity on asparagine residues in peptides or proteins. (canada.ca)
  • Remove-iT PNGase F is an amidase, which cleaves between the innermost GlcNAc and asparagine residues of high mannose, hybrid, and complex oligosaccharides from N-linked glycoproteins. (neb.com)
  • The active site of serum paraoxonase-1 may also utilise 2 histidine residues, glutamic acid and asparagine to stabilise transitional sites. (prospecbio.com)
  • Specific enzymes (glycosyltransferases, GTs), ubiquitous in all domains of life, execute an enzymatic reaction, transferring either simple monosaccharides or polysaccharides en bloc via covalent linkage to OH groups (O-glycosylation on serine or threonine residues) or to NH2-groups (N-glycosylation on asparagine) by O-GTs or N-GTs, respectively. (unige.ch)
  • In addition to being a component of proteins, asparagine helps to break down toxic ammonia within cells, is important for protein modification, and is needed for making a molecule that transmits signals in the brain (a neurotransmitter). (medlineplus.gov)
  • Asparagine attaches sugars to proteins. (editthis.info)
  • Dr. Locher's research focuses on the structure and mechanism of integral membrane proteins catalyzing active transport reactions of protein N-glycosylation. (nih.gov)
  • Our understanding of the reaction mechanisms of membrane-embedded transport proteins or enzymes is insufficient. (nih.gov)
  • o Phosphorus is a constituent of cell membranes, certain proteins, all nucleic acids and nucleotides, and is required for all phosphorylation reactions. (sciencegajab.com)
  • Recently we reported that CHB11-1-3, a Chinese hamster ovary cell mutant defective in glycosylation of asparagine-linked proteins, is defective in the synthesis of dolichol [Quellhorst et al. (nih.gov)
  • This chemical reaction also produces another form of galactose (UDP-galactose) that is used to build galactose-containing proteins and fats. (nih.gov)
  • This involves a reaction between an aldehyde containing molecule and one with either asparagine or glutamine at the N-terminal, resulting in formation of a bioconjugate product. (shu.edu)
  • The most common compounds involved as a donor/acceptor pair in transamination reactions are glutamate and α-KG, which participate in reactions with many different aminotransferases. (tdmuv.com)
  • Thus, after being released in, and channeled from, the glutaminase site, the ammonia molecule attacks the bound βAspAMP 1 to give asparagine and AMP via a tetrahedral intermediate. (wikipedia.org)
  • Ammonia the highly toxic product of protein catabolism, is rapidly inactivated by a variety of reactions. (tdmuv.com)
  • When certain foods are cooked at high temperatures, sugars, such as glucose and fructose, can react with the free amino acid, asparagine, to form acrylamide. (nih.gov)
  • Recent findings, propelled by UK scientist Professor Don Mottram at the University of Reading,suggest that the free amino acid asparagine, found naturally in potatoes and cereals, could play akey role in the formation of acrylamide. (foodnavigator-usa.com)
  • Using an antioxidant-rich extracts from bamboo leaf and green tea could reduce the formation of acrylamide in an asparagine-glucose model system heated by microwave, according to a new study. (foodnavigator.com)
  • The new study used a model asparagine-glucose system, with or without the bamboo leaves and green tea extracts on the formation of acrylamide when the mixture was heated at 180 degrees Celsius by microwave. (foodnavigator.com)
  • 3-aminopropionamide, a transient intermediate formed via decarboxylating reaction of asparagine, is an important and direct precursor contributing to the formation of acrylamide. (foodnavigator.com)
  • New data from Vancouver-based Functional Technologies indicates that testing by a third part revealed that the acrylamide-preventing yeast technology may reduce levels of asparagine - the main known precursor in the formation of acrylamide - by approximately 85% within 30 minutes. (foodnavigator-usa.com)
  • It is known however that antioxidants can in-hibit lipid oxidation and by extension lower the formation of acrylamide, since some lipid oxidation products such as acrolein might react with asparagine. (slu.se)
  • Amino and amide groups from these 2 substances are freely transferred to other carbon skeletons by transamination and transamidation reactions. (tdmuv.com)
  • Due to the transient response from these susceptible cancers in reaction to the asparagine depletion, tumor growth is significantly inhibited due to nutritional deficiency. (wikipedia.org)
  • Tumor cell proliferation is blocked because of the interruption of asparagine-dependent protein synthesis. (prospecbio.com)
  • The reason it's used is that so many tumor cells depend on a high concentration of asparagine in the blood. (prospecbio.com)
  • Serious hypersensitivity reactions to RYLAZE, including anaphylaxis. (nih.gov)
  • Toxic reactions are generally hypersensitivity reactions or depression of protein synthesis. (nih.gov)
  • Discontinue ASPARLAS in patients with serious hypersensitivity reactions. (nih.gov)
  • Depletion of the essential amino acid L-Asparagine results in rapid inhibition of protein synthesis. (thehematologist.org)
  • Acrylamide is formed as a reaction product between asparagine and reducing sugars when certain foods are baked or fried at temperatures exceeding 120oC. (canada.ca)
  • When heated, the amino acid asparagine enters a chemical reaction. (prospecbio.com)
  • Thus, it can't take part in the chemical reaction, which means it can't produce carcinogens like acrylamide. (prospecbio.com)
  • As a result, asparagine is not available anymore for the chemical reaction that forms acrylamide when carbohydrate-containing foods, such as bread, cake, cookies, potato chips and cereals, are being heated. (bakeryandsnacks.com)
  • N-glycans can be released by glycosidases, whereas O-glycans are often cleaved by chemical reaction. (nih.gov)
  • All paraoxonase enzymes use catalytic calcium ions as an oxy-anion to secure substrates and stabilise reactions. (prospecbio.com)
  • Enzymes can speed reaction by a factor of 10^20. (editthis.info)
  • The transient presence or absence of this particular interaction can influence key outcomes in enzymes, such as the reaction rate or selectivity. (aiche.org)
  • A nested polymerase chain reaction was used to amplify a fragment of the gene for dihydrofolate reductase of Plasmodium falciparum containing codon 108, where a point mutation, causing a serine to asparagine change, occurs in pyrimethamine resistant parasites. (nih.gov)
  • Therefore, ideally O-GT-based reactions should be also be soluble, yet most donor sugars and enzymatic reactions are executed by (naturally) membrane-linked O-GTs. (unige.ch)
  • Hydroxylation and dehydroxylation of amino acid Asparagine. (ed.ac.uk)
  • However, in a study titled " Asparagine hydroxylation is a reversible post-translational modification " which was recently published in the journal Molecular & Cellular Proteomics, IGMM scientists provided data indicating that asparagine hydroxylation is reversible. (ed.ac.uk)
  • Immune signaling in early-stage COVID-19, regulating translation on the ER membrane and reversing asparagine hydroxylation. (asbmb.org)
  • Free asparagine accumulates in cereals when there is an excess of nitrogen and at the same time a deficit of sulphur. (slu.se)
  • Some product of these reactions are utilized for other purposes (thus salvaging a portion of the amino nitrogen), while others are excreted. (tdmuv.com)
  • This reaction is essential in eukaryotes and implicated in processes such as protein folding and quality control, organism development, or host-pathogen interactions. (nih.gov)
  • The second half of the lecture deals with OST catalyzing asparagine-linked (N-linked) protein glycosylation. (nih.gov)
  • It has been shown that free asparagine is the determining factor for acrylamide formation in cereals. (slu.se)
  • Proper fertilising and selection of crops with low levels of free asparagine might be the key to lower acrylamide formation in oat products. (slu.se)
  • Antioxidants might inhibit acrylamide formation, but some studies have found the opposite and theorises that some antioxidants prevent further reaction of acrylamide with other compounds. (slu.se)
  • New data from Functional Technologies now indicates that the company's proprietary acrylamide-preventing (AP) yeast may reduce asparagine levels to near non-detectable levels within 60 minutes when used in food application that traditionally do not incorporate yeast ingredients as processing aids. (foodnavigator-usa.com)
  • Longer fermentation time of yeast doughs have been shown to produce bread with a lower acrylamide level since the yeast consumes the free asparagine. (slu.se)
  • Sourdough as leavening agent is not as effective at consuming asparagine, probably due to compe-tition from bacteria that impairs the yeast in the sourdough. (slu.se)
  • Acrylamide is a suspected carcinogen that is formed during by heat-induced reaction between sugar and an amino acid called asparagine. (foodnavigator-usa.com)
  • The calcium ion that lies deepest in the tunnel has a structural role, while the other calcium ion is involved in catalysing reactions and positioning the substrate. (prospecbio.com)
  • The most common (incidence ≥10%) grade ≥3 adverse reactions were elevated transaminase, bilirubin increased, pancreatitis, and abnormal clotting studies. (nih.gov)
  • Parasites in the samples taken at 3 weeks carried only the asparagine mutant. (nih.gov)
  • The Locher group has determined several crystal structures that have helped elucidate the reaction mechanisms of ABC transporters and oligosaccharyltransferase, including the first structures of an ABC exporter and type I and type II ABC importers as well as the first structure of a complete bacterial oligosaccharyltransferase. (nih.gov)
  • This phenomenon is also referred to as one of the non-enzymatic browning reactions. (novozymes.com)
  • However, it is challenging to combine these enzymatic and chemical reactions in order to analyze both N- and O-glycans. (nih.gov)
  • These reactions, although important for cellular physiology, are generally not well understood at the molecular level because (i) high-resolution structures are scarce, and (ii) functional in vitro assays are challenging to establish. (nih.gov)